A term used to describe a variety of localized asymmetrical SKIN thickening that is similar to those of SYSTEMIC SCLERODERMA but without the disease features in the multiple internal organs and BLOOD VESSELS. Lesions may be characterized as patches or plaques (morphea), bands (linear), or nodules.
A chronic multi-system disorder of CONNECTIVE TISSUE. It is characterized by SCLEROSIS in the SKIN, the LUNGS, the HEART, the GASTROINTESTINAL TRACT, the KIDNEYS, and the MUSCULOSKELETAL SYSTEM. Other important features include diseased small BLOOD VESSELS and AUTOANTIBODIES. The disorder is named for its most prominent feature (hard skin), and classified into subsets by the extent of skin thickening: LIMITED SCLERODERMA and DIFFUSE SCLERODERMA.
A rapid onset form of SYSTEMIC SCLERODERMA with progressive widespread SKIN thickening over the arms, the legs and the trunk, resulting in stiffness and disability.
The least progressive form of SYSTEMIC SCLERODERMA with skin thickening restricted to the face, neck and areas distal to the elbows and/or knees, sparing the trunk. The CREST SYNDROME is a form of limited scleroderma.
An idiopathic vascular disorder characterized by bilateral Raynaud phenomenon, the abrupt onset of digital paleness or CYANOSIS in response to cold exposure or stress.
The outer covering of the body that protects it from the environment. It is composed of the DERMIS and the EPIDERMIS.
The noninvasive microscopic examination of the microcirculation, commonly done in the nailbed or conjunctiva. In addition to the capillaries themselves, observations can be made of passing blood cells or intravenously injected substances. This is not the same as endoscopic examination of blood vessels (ANGIOSCOPY).
Permanent dilation of preexisting blood vessels (CAPILLARIES; ARTERIOLES; VENULES) creating small focal red lesions, most commonly in the skin or mucous membranes. It is characterized by the prominence of skin blood vessels, such as vascular spiders.
The thin, horny plates that cover the dorsal surfaces of the distal phalanges of the fingers and toes of primates.
Connective tissue cells which secrete an extracellular matrix rich in collagen and other macromolecules.
A layer of vascularized connective tissue underneath the EPIDERMIS. The surface of the dermis contains innervated papillae. Embedded in or beneath the dermis are SWEAT GLANDS; HAIR FOLLICLES; and SEBACEOUS GLANDS.
Antibodies that react with self-antigens (AUTOANTIGENS) of the organism that produced them.
Autoantibodies directed against various nuclear antigens including DNA, RNA, histones, acidic nuclear proteins, or complexes of these molecular elements. Antinuclear antibodies are found in systemic autoimmune diseases including systemic lupus erythematosus, Sjogren's syndrome, scleroderma, polymyositis, and mixed connective tissue disease.
Any pathological condition where fibrous connective tissue invades any organ, usually as a consequence of inflammation or other injury.
A mild form of LIMITED SCLERODERMA, a multi-system disorder. Its features include symptoms of CALCINOSIS; RAYNAUD DISEASE; ESOPHAGEAL MOTILITY DISORDERS; sclerodactyly, and TELANGIECTASIS. When the defect in esophageal function is not prominent, it is known as CRST syndrome.
The most common form of fibrillar collagen. It is a major constituent of bone (BONE AND BONES) and SKIN and consists of a heterotrimer of two alpha1(I) and one alpha2(I) chains.
A DNA-binding protein that interacts with a 17-base pair sequence known as the CENP-B box motif. The protein is localized constitutively to the CENTROMERE and plays an important role in its maintenance.
A syndrome characterized by slowly progressive unilateral atrophy of facial subcutaneous fat, muscle tissue, skin, cartilage, and bone. The condition typically progresses over a period of 2-10 years and then stabilizes.
A diverse group of lung diseases that affect the lung parenchyma. They are characterized by an initial inflammation of PULMONARY ALVEOLI that extends to the interstitium and beyond leading to diffuse PULMONARY FIBROSIS. Interstitial lung diseases are classified by their etiology (known or unknown causes), and radiological-pathological features.
A heterogeneous group of disorders, some hereditary, others acquired, characterized by abnormal structure or function of one or more of the elements of connective tissue, i.e., collagen, elastin, or the mucopolysaccharides.
A complex of related glycopeptide antibiotics from Streptomyces verticillus consisting of bleomycin A2 and B2. It inhibits DNA metabolism and is used as an antineoplastic, especially for solid tumors.
Leisure activities engaged in for pleasure.
A factor synthesized in a wide variety of tissues. It acts synergistically with TGF-alpha in inducing phenotypic transformation and can also act as a negative autocrine growth factor. TGF-beta has a potential role in embryonal development, cellular differentiation, hormone secretion, and immune function. TGF-beta is found mostly as homodimer forms of separate gene products TGF-beta1, TGF-beta2 or TGF-beta3. Heterodimers composed of TGF-beta1 and 2 (TGF-beta1.2) or of TGF-beta2 and 3 (TGF-beta2.3) have been isolated. The TGF-beta proteins are synthesized as precursor proteins.
A skin ulcer is a breakdown of the skin's surface and underlying tissues, often caused by prolonged pressure, infection, or poor circulation, leading to a loss of continuity in the epidermis and dermis, potentially extending into deeper layers such as subcutaneous tissue, muscle, and bone.
A CCN protein family member that regulates a variety of extracellular functions including CELL ADHESION; CELL MIGRATION; and EXTRACELLULAR MATRIX synthesis. It is found in hypertrophic CHONDROCYTES where it may play a role in CHONDROGENESIS and endochondral ossification.
A polypeptide substance comprising about one third of the total protein in mammalian organisms. It is the main constituent of SKIN; CONNECTIVE TISSUE; and the organic substance of bones (BONE AND BONES) and teeth (TOOTH).
A process in which normal lung tissues are progressively replaced by FIBROBLASTS and COLLAGEN causing an irreversible loss of the ability to transfer oxygen into the bloodstream via PULMONARY ALVEOLI. Patients show progressive DYSPNEA finally resulting in death.
Disorders that are characterized by the production of antibodies that react with host tissues or immune effector cells that are autoreactive to endogenous peptides.
Increased VASCULAR RESISTANCE in the PULMONARY CIRCULATION, usually secondary to HEART DISEASES or LUNG DISEASES.
Disorders of connective tissue, especially the joints and related structures, characterized by inflammation, degeneration, or metabolic derangement.
Photochemotherapy using PSORALENS as the photosensitizing agent and ultraviolet light type A (UVA).
Pathological processes in the ESOPHAGUS.
Endogenous tissue constituents that have the ability to interact with AUTOANTIBODIES and cause an immune response.
A broad family of synthetic organosiloxane polymers containing a repeating silicon-oxygen backbone with organic side groups attached via carbon-silicon bonds. Depending on their structure, they are classified as liquids, gels, and elastomers. (From Merck Index, 12th ed)
A syndrome with overlapping clinical features of systemic lupus erythematosus, scleroderma, polymyositis, and Raynaud's phenomenon. The disease is differentially characterized by high serum titers of antibodies to ribonuclease-sensitive extractable (saline soluble) nuclear antigen and a "speckled" epidermal nuclear staining pattern on direct immunofluorescence.
A receptor-regulated smad protein that undergoes PHOSPHORYLATION by ACTIVIN RECEPTORS, TYPE I. Activated Smad3 can bind directly to DNA, and it regulates TRANSFORMING GROWTH FACTOR BETA and ACTIVIN signaling.
A congenital defect in which the mouth is unusually small. (Dorland, 27th ed)
Ocular disorders attendant upon non-ocular disease or injury.
DNA TOPOISOMERASES that catalyze ATP-independent breakage of one of the two strands of DNA, passage of the unbroken strand through the break, and rejoining of the broken strand. DNA Topoisomerases, Type I enzymes reduce the topological stress in the DNA structure by relaxing the superhelical turns and knotted rings in the DNA helix.
'Skin diseases' is a broad term for various conditions affecting the skin, including inflammatory disorders, infections, benign and malignant tumors, congenital abnormalities, and degenerative diseases, which can cause symptoms such as rashes, discoloration, eruptions, lesions, itching, or pain.
The clear constricted portion of the chromosome at which the chromatids are joined and by which the chromosome is attached to the spindle during cell division.
Skin diseases characterized by local or general distributions of blisters. They are classified according to the site and mode of blister formation. Lesions can appear spontaneously or be precipitated by infection, trauma, or sunlight. Etiologies include immunologic and genetic factors. (From Scientific American Medicine, 1990)
The amount of a gas taken up, by the pulmonary capillary blood from the alveolar gas, per minute per unit of average pressure of the gradient of the gas across the BLOOD-AIR BARRIER.
The larger of the two terminal branches of the brachial artery, beginning about one centimeter distal to the bend of the elbow. Like the RADIAL ARTERY, its branches may be divided into three groups corresponding to their locations in the forearm, wrist, and hand.
3-Mercapto-D-valine. The most characteristic degradation product of the penicillin antibiotics. It is used as an antirheumatic and as a chelating agent in Wilson's disease.
Cells propagated in vitro in special media conducive to their growth. Cultured cells are used to study developmental, morphologic, metabolic, physiologic, and genetic processes, among others.
The part of the face above the eyes.
The minute vessels that connect the arterioles and venules.
Historically, a heterogeneous group of acute and chronic diseases, including rheumatoid arthritis, systemic lupus erythematosus, progressive systemic sclerosis, dermatomyositis, etc. This classification was based on the notion that "collagen" was equivalent to "connective tissue", but with the present recognition of the different types of collagen and the aggregates derived from them as distinct entities, the term "collagen diseases" now pertains exclusively to those inherited conditions in which the primary defect is at the gene level and affects collagen biosynthesis, post-translational modification, or extracellular processing directly. (From Cecil Textbook of Medicine, 19th ed, p1494)
Implants used to reconstruct and/or cosmetically enhance the female breast. They have an outer shell or envelope of silicone elastomer and are filled with either saline or silicone gel. The outer shell may be either smooth or textured.
Scalp dermatoses refer to various inflammatory skin conditions affecting the scalp, including seborrheic dermatitis, psoriasis, atopic dermatitis, and tinea capitis, often characterized by symptoms such as redness, scaling, itching, and hair loss.
A chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys, and serosal membranes. It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow.
A state in south central Australia. Its capital is Adelaide. It was probably first visited by F. Thyssen in 1627. Later discoveries in 1802 and 1830 opened up the southern part. It became a British province in 1836 with this self-descriptive name and became a state in 1901. (From Webster's New Geographical Dictionary, 1988, p1135)
Diseases in any segment of the GASTROINTESTINAL TRACT from ESOPHAGUS to RECTUM.
An inhibitory smad protein that associates with TRANSFORMING GROWTH FACTOR BETA RECEPTORS and BONE MORPHOGENETIC PROTEIN RECEPTORS. It negatively regulates SIGNAL TRANSDUCTION PATHWAYS by inhibiting PHOSPHORYLATION of RECEPTOR-REGULATED SMAD PROTEINS.
A pathological process consisting of hardening or fibrosis of an anatomical structure, often a vessel or a nerve.
Pathological processes involving any part of the LUNG.
A subacute or chronic inflammatory disease of muscle and skin, marked by proximal muscle weakness and a characteristic skin rash. The illness occurs with approximately equal frequency in children and adults. The skin lesions usually take the form of a purplish rash (or less often an exfoliative dermatitis) involving the nose, cheeks, forehead, upper trunk, and arms. The disease is associated with a complement mediated intramuscular microangiopathy, leading to loss of capillaries, muscle ischemia, muscle-fiber necrosis, and perifascicular atrophy. The childhood form of this disease tends to evolve into a systemic vasculitis. Dermatomyositis may occur in association with malignant neoplasms. (From Adams et al., Principles of Neurology, 6th ed, pp1405-6)
The use of ultraviolet electromagnetic radiation in the treatment of disease, usually of the skin. This is the part of the sun's spectrum that causes sunburn and tanning. Ultraviolet A, used in PUVA, is closer to visible light and less damaging than Ultraviolet B, which is ionizing.
Measurement of the various processes involved in the act of respiration: inspiration, expiration, oxygen and carbon dioxide exchange, lung volume and compliance, etc.
Fluid accumulation within the PERICARDIUM. Serous effusions are associated with pericardial diseases. Hemopericardium is associated with trauma. Lipid-containing effusion (chylopericardium) results from leakage of THORACIC DUCT. Severe cases can lead to CARDIAC TAMPONADE.

Positive IgG Western blot for Borrelia burgdorferi in Colombia. (1/180)

In order to evaluate the presence of specific IgG antibodies to Borrelia burgdorferi in patients with clinical manifestations associated with Lyme borreliosis in Cali, Colombia, 20 serum samples from patients with dermatologic signs, one cerebrospinal fluid (CSF) sample from a patient with chronic neurologic and arthritic manifestations, and twelve serum samples from individuals without clinical signs associated with Lyme borreliosis were analyzed by IgG Western blot. The results were interpreted following the recommendations of the Centers for Diseases Control and Prevention (CDC) for IgG Western blots. Four samples fulfilled the CDC criteria: two serum specimens from patients with morphea (localized scleroderma), the CSF from the patient with neurologic and arthritic manifestations, and one of the controls. Interpretation of positive serology for Lyme disease in non-endemic countries must be cautious. However these results suggest that the putative "Lyme-like" disease may correlate with positivity on Western blots, thus raising the possibility that a spirochete genospecies distinct from B. burgdorferi sensu stricto, or a Borrelia species other than B. burgdorferi sensu lato is the causative agent. Future work will focus on a survey of the local tick and rodent population for evidence of spirochete species that could be incriminated as the etiologic agent.  (+info)

Expression of type XVI collagen in human skin fibroblasts: enhanced expression in fibrotic skin diseases. (2/180)

Abundance of type XVI collagen mRNA in normal human dermal fibroblasts explanted from different horizontal layers was determined using RNase protection assays. Type XVI collagen mRNA level in the fibroblasts explanted from the upper dermis was greater than those of the middle and lower dermis. The antibody raised against the synthetic N-terminal noncollagenous region reacted with approximately 210 kDa collagenous polypeptide in the culture medium of fibroblasts. Immunohistochemical study of normal human skin demonstrated that the antibody reacted preferentially with the fibroblasts and the extracellular matrix in the upper dermis rather than those in the middle and lower dermis. Type XVI collagen mRNA level was elevated 2.3-fold in localized scleroderma and 3.6-fold in systemic scleroderma compared with keloid and normal controls. Immunofluorescent study revealed that an intense immunoreactivity with the antibody was observed in the upper to lower dermal matrix and fibroblasts in the skin of systemic scleroderma as compared with normal skin. The results suggest that expression of type XVI collagen, a member of fibril-associated collagens with interrupted triple helices, in human skin fibroblasts can be heterogeneous in the dermal layers and can be modulated by some fibrotic diseases.  (+info)

Effect of superoxide dismutase on bleomycin-induced dermal sclerosis: implications for the treatment of systemic sclerosis. (3/180)

Bleomycin has a chemical toxicity capable of inducing superoxide radicals, which are suggested to play an important part in bleomycin-induced pulmonary fibrosis. We have recently established a mouse model for scleroderma induced by repeated local injections of bleomycin. In this study, we examined the inhibitory effect of superoxide dismutase on the development of dermal sclerosis induced by bleomycin using this mouse model. PC-superoxide dismutase, which is a lecithinized superoxide dismutase with high tissue accumulation and long half-life in blood, was administered (3000 U per kg; dissolved in 5% mannitol) 3 h before the injection of bleomycin in C3H mice for 3 wk. Systemic PC-superoxide dismutase markedly inhibited the development of dermal sclerosis, which was also accompanied by a decrease in the number of infiltrating mast cells and eosinophils. Furthermore, the hydroxyproline content in the skin was significantly reduced, as compared with mice treated with bleomycin only or bleomycin and 5% mannitol. In a separate experiment, after the development of dermal sclerosis following treatment with bleomycin for 3 wk, PC-superoxide dismutase was administered for 2 wk. Histologic examination again revealed a reduction of dermal sclerosis, followed by a significant associate in the number of both mast cells and eosinophils. The hydroxyproline content in the skin was not significantly decreased, however, even after injections of high amounts of PC-superoxide dismutase (30,000 U per kg). These results support the involvement of oxygen free radicals in bleomycin-induced dermal sclerosis, and also indicate that administration of superoxide dismutase may be effective in the therapeutic approach in systemic sclerosis.  (+info)

Autoantibodies to the extracellular matrix microfibrillar protein, fibrillin 1, in patients with localized scleroderma. (4/180)

OBJECTIVE: Serum autoantibodies to fibrillin 1, the major component of microfibrils in the extracellular matrix, recently have been reported to occur in the tight skin mouse and in patients with systemic sclerosis, but not in patients with other connective tissue diseases. This study was undertaken to determine whether antifibrillin 1 antibodies could be detected in patients with localized forms of scleroderma. METHODS: Sera from 50 patients with localized scleroderma (27 with linear scleroderma and 23 with morphea) and 51 normal controls were tested for IgG and IgM antifibrillin 1 autoantibodies, using a radioimmunoassay (RIA) and a human recombinant fibrillin 1 protein (rFbn-1). RESULTS: Both in patients with linear scleroderma and in those with morphea, mean levels of IgM and IgG binding to rFbn-1 were significantly higher than in controls. Eight patients with linear scleroderma (30%) and 6 patients with morphea (26%) had IgG autoantibodies to fibrillin 1 (rFbn-1) by RIA, compared with 3 controls (6%) (P = 0.006 and P = 0.022, respectively). No correlations between antifibrillin 1 antibodies and active skin disease or antinuclear antibody positivity were found. CONCLUSION: Autoantibodies to fibrillin 1 occur in patients with both forms of localized scleroderma (linear scleroderma and morphea). The clinical and pathogenetic significance of this autoimmune response remains to be determined.  (+info)

Systemic sclerosis sine scleroderma: demographic, clinical, and serologic features and survival in forty-eight patients. (5/180)

OBJECTIVE: To describe the demographic, clinical, and laboratory features and natural history of patients with systemic sclerosis sine scleroderma (ssSSc), and to compare them with those of patients with SSc and limited cutaneous involvement (IcSSc). METHODS: The University of Pittsburgh Scleroderma Databank served as the data source. Patients were divided into those who had no skin thickening (ssSSc) and those who had skin thickening only distal to elbows or knees and/or of the face (IcSSc) during their disease course. These two groups were compared with regard to demographic characteristics, clinical, laboratory, and serologic features, and survival rates. Chi-square and Student's t-test analyses were performed, and Fisher's exact test was used as appropriate. RESULTS: Of 555 consecutive patients without diffuse cutaneous SSc, 48 (9%) had ssSSc and 507 (91%) had IcSSc. The ssSSc patients had a mean total disease duration of 18.6 years (15.1 years before study entry and 3.5 years of followup after study entry), and had not developed IcSSc or another connective tissue disease. Other than the absence of skin thickening, the ssSSc group had no significant differences in individual internal organ involvements, laboratory features, serum autoantibody type, or survival rate compared with patients with IcSSc. Within the category of lung involvement, patients with ssSSc had a significantly greater frequency of dyspnea with mild exertion or at rest, and a tendency toward reduced carbon monoxide diffusing capacity (<70% of predicted normal) and primary pulmonary arterial hypertension. Patients with IcSSc had significantly more frequent individual manifestations of digital pitting scars, digital-tip ulcers, telangiectasia, and calcinosis than those with ssSSc, in part related to increased time of observation. Puffy fingers and finger joint contractures were detected significantly more often in IcSSc patients. CONCLUSION: Systemic sclerosis sine scleroderma should be included in the spectrum of SSc with limited cutaneous involvement and should not be considered a distinct or separate disorder.  (+info)

Hereditary deficiency of the seventh component of complement. (6/180)

Deficiency of the seventh component of complement has been found in the serum of a 42-yr-old Caucasian woman who has Raynaud's phenomenon, sclerodactyly, and telangiectasia. Partial deficiency was found in the serum of the patient's parents and children, indicating a pattern of inheritance of autosomal codominance. Transfusion experiments indicated that exogenous C7 had a 91-h halk-life in the patient. There was no evidence for C7 synthesis after transfusion. No C7 inhibitors were detected in the patient's serum. The patient's serum was found to support the activation of complement by both the classical and properdin pathways to the C7 stage. The addition of C7 to the patient's serum permitted it to support hemolytic reactions initiated by either pathway. No defects could be detected in plasma or whole blood coagulation. The patient's serum was deficient in opsonizing unsensitized yeast particles in serum and in the generation of chemotactic factor by antigen-antibody complexes and endotoxin. Both deficiencies were corrected by the addition of C7. These observations suggest a key role for C7 for in vitro yeast phagocytosis and chemotaxis generation. However, the patient's lack of infections indicates a relatively minor role for C7 in human resistance to infection.  (+info)

Circulating collagen metabolites in systemic sclerosis. Differences between limited and diffuse form and relationship with pulmonary involvement. (7/180)

OBJECTIVE: To study collagen metabolites in systemic sclerosis (SSc) and their relationship with clinical manifestations of the disease. METHODS: Forty-eight SSc patients, 13 with a diffuse form (dcSSc), 23 with a limited form (lcSSc) and 12 with suspected SSc not fulfilling the ACR criteria, and 31 healthy controls were examined. Serum concentrations of aminoterminal type III procollagen peptide (PIIINP), aminoterminal and carboxyterminal type I procollagen peptides (PINP and PICP) and cross-linked carboxyterminal telopeptide of collagen I (ICTP) were determined by radioimmunoassay. RESULTS: Increased serum concentrations of ICTP were found in SSc patients compared with controls. Distinctly higher levels of ICTP were observed in dcSSc than in lcSSc. High serum ICTP was correlated with skin score and acute phase reactants, and with reduced pulmonary function. Serum PIIINP concentration was elevated in both lcSSc and dcSSc. CONCLUSION: Augmented collagen catabolism accompanies the increased collagen synthesis in SSc. Serum ICTP concentration is a marker of this feature and also reflects clinical severity.  (+info)

Accuracy of alternative approaches to capture-recapture estimates of disease frequency: internal validity analysis of data from five sources. (8/180)

The authors used "internal validity analysis" to evaluate the performance of various capture-recapture methods. Data from studies with five overlapping, incomplete lists generated subgroups whose known sizes were compared with estimates derived from various four-source capture-recapture analyses. In 15 data sets unanalyzed previously (five subgroups of each of three new studies), the authors observed a trend toward mean underestimation of the known population size by 16-25%. (Coverage of the 90% confidence intervals associated with the method found to be optimal was acceptable (13/15), despite the downward bias.) The authors conjectured that (with the obvious exception of geographically disparate lists) most data sets used by epidemiologists tend to have a net positive dependence; that is, cases captured by one source are more likely to be captured by some other available source than are cases selected randomly from the population, and this trend results in a bias toward underestimation. Attempts to ensure that the underlying assumptions of the methods are met, such as minimizing (or adjusting adequately) for the possibility of loss due to death or migration, as was undertaken in one exceptional study, appear likely to improve the behavior of these methods.  (+info)

Localized scleroderma, also known as morphea, is a rare autoimmune disorder that affects the skin and connective tissues. It is characterized by thickening and hardening (sclerosis) of the skin in patches or bands, usually on the trunk, limbs, or face. Unlike systemic scleroderma, localized scleroderma does not affect internal organs, although it can cause significant disfigurement and disability in some cases.

There are two main types of localized scleroderma: plaque morphea and generalized morphea. Plaque morphea typically presents as oval or circular patches of thickened, hard skin that are often white or pale in the center and surrounded by a purple or darker border. Generalized morphea, on the other hand, is characterized by larger areas of sclerosis that can cover much of the body surface.

The exact cause of localized scleroderma is not fully understood, but it is thought to involve an overactive immune system response that leads to inflammation and scarring of the skin and underlying tissues. Treatment typically involves a combination of topical therapies (such as corticosteroids or calcineurin inhibitors), phototherapy, and systemic medications (such as methotrexate or mycophenolate mofetil) in more severe cases.

Systemic Scleroderma, also known as Systemic Sclerosis (SSc), is a rare, chronic autoimmune disease that involves the abnormal growth and accumulation of collagen in various connective tissues, blood vessels, and organs throughout the body. This excessive collagen production leads to fibrosis or scarring, which can cause thickening, hardening, and tightening of the skin and damage to internal organs such as the heart, lungs, kidneys, and gastrointestinal tract.

Systemic Scleroderma is characterized by two main features: small blood vessel abnormalities (Raynaud's phenomenon) and fibrosis. The disease can be further classified into two subsets based on the extent of skin involvement: limited cutaneous systemic sclerosis (lcSSc) and diffuse cutaneous systemic sclerosis (dcSSc).

Limited cutaneous systemic sclerosis affects the skin distally, typically involving fingers, hands, forearms, feet, lower legs, and face. It is often associated with Raynaud's phenomenon, calcinosis, telangiectasias, and pulmonary arterial hypertension.

Diffuse cutaneous systemic sclerosis involves more extensive skin thickening and fibrosis that spreads proximally to affect the trunk, upper arms, thighs, and face. It is commonly associated with internal organ involvement, such as interstitial lung disease, heart disease, and kidney problems.

The exact cause of Systemic Scleroderma remains unknown; however, it is believed that genetic, environmental, and immunological factors contribute to its development. There is currently no cure for Systemic Scleroderma, but various treatments can help manage symptoms, slow disease progression, and improve quality of life.

Diffuse scleroderma is a medical condition that falls under the systemic sclerosis category of autoimmune rheumatic diseases. It is characterized by thickening and hardening (sclerosis) of the skin and involvement of internal organs. In diffuse scleroderma, the process affects extensive areas of the skin and at least one internal organ.

The disease process involves an overproduction of collagen, a protein that makes up connective tissues in the body. This excessive collagen deposition leads to fibrosis (scarring) of the skin and various organs, including the esophagus, gastrointestinal tract, heart, lungs, and kidneys.

Diffuse scleroderma can present with a rapid progression of skin thickening within the first few years after onset. The skin involvement may extend to areas beyond the hands, feet, and face, which are commonly affected in limited scleroderma (another form of systemic sclerosis). Additionally, patients with diffuse scleroderma have a higher risk for severe internal organ complications compared to those with limited scleroderma.

Early diagnosis and appropriate management of diffuse scleroderma are crucial to prevent or slow down the progression of organ damage. Treatment typically involves a multidisciplinary approach, focusing on symptom management, immunosuppressive therapy, and addressing specific organ involvement.

Limited scleroderma, also known as limited cutaneous systemic sclerosis (lcSSc), is a subtype of scleroderma, a chronic autoimmune connective tissue disease. In this form, the fibrosis or hardening and thickening of the skin is generally limited to areas below the elbows and knees, as well as the face and neck.

The limited cutaneous form often involves the hands, causing a tightening of the skin on the fingers, known as "sclerodactyly." It can also affect the internal organs, but this is usually less severe and occurs later in the disease course compared to diffuse scleroderma.

A common characteristic of limited scleroderma is the presence of CREST syndrome, an acronym for Calcinosis, Raynaud's phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia. These are specific symptoms associated with this subtype.

However, it is important to note that the manifestations of scleroderma can vary significantly from person to person, and not everyone with limited scleroderma will develop all the features of CREST syndrome.

Raynaud's disease, also known as Raynaud's phenomenon or syndrome, is a condition that affects the blood vessels, particularly in the fingers and toes. It is characterized by episodes of vasospasm (constriction) of the small digital arteries and arterioles, which can be triggered by cold temperatures or emotional stress. This results in reduced blood flow to the affected areas, causing them to become pale or white and then cyanotic (blue) due to the accumulation of deoxygenated blood. As the episode resolves, the affected areas may turn red as blood flow returns, sometimes accompanied by pain, numbness, or tingling sensations.

Raynaud's disease can be primary, meaning it occurs without an underlying medical condition, or secondary, which is associated with connective tissue disorders, autoimmune diseases, or other health issues such as carpal tunnel syndrome, vibration tool usage, or smoking. Primary Raynaud's is more common and tends to be less severe than secondary Raynaud's.

Treatment for Raynaud's disease typically involves avoiding triggers, keeping the body warm, and using medications to help dilate blood vessels and improve circulation. In some cases, lifestyle modifications and smoking cessation may also be recommended to manage symptoms and prevent progression of the condition.

In medical terms, the skin is the largest organ of the human body. It consists of two main layers: the epidermis (outer layer) and dermis (inner layer), as well as accessory structures like hair follicles, sweat glands, and oil glands. The skin plays a crucial role in protecting us from external factors such as bacteria, viruses, and environmental hazards, while also regulating body temperature and enabling the sense of touch.

Microscopic angioscopy is not a widely recognized or established medical term. However, based on the individual terms, it can be interpreted as the use of a microscope with an angioscope (a type of endoscope used for visualizing the interior of blood vessels) to examine the microscopic structures of the inner walls of blood vessels. This technique would allow for detailed examination of the vasculature at a cellular level, potentially providing valuable information for research and diagnosis of various vascular diseases. However, as this is not a standard medical procedure or term, it's essential to consult the relevant literature or experts in the field for more precise information.

Telangiectasia is a medical term that refers to the dilation and widening of small blood vessels called capillaries, leading to their visibility under the skin or mucous membranes. These dilated vessels often appear as tiny red lines or patterns, measuring less than 1 millimeter in diameter.

Telangiectasias can occur in various parts of the body, such as the face, nose, cheeks, legs, and fingers. They are typically harmless but may cause cosmetic concerns for some individuals. In certain cases, telangiectasias can be a sign of an underlying medical condition, like rosacea, hereditary hemorrhagic telangiectasia (HHT), or liver disease.

It is essential to consult with a healthcare professional if you notice any unusual changes in your skin or mucous membranes, as they can provide appropriate evaluation and treatment recommendations based on the underlying cause of the telangiectasias.

In the context of medical terminology, "nails" primarily refer to the keratinous plates that are found at the tips of fingers and toes. These specialized structures are part of the outermost layer of the skin (epidermis) and are formed by a type of cells called keratinocytes. The nails serve to protect the delicate underlying tissues from trauma, and they also aid in tasks such as picking up small objects or scratching itches.

The medical term for fingernails and toenails is "unguis," which comes from Latin. Each nail consists of several parts:

1. Nail plate: The visible part of the nail that is hard and flat, made up of keratin.
2. Nail bed: The skin beneath the nail plate to which the nail plate is attached; it supplies blood to the nail.
3. Matrix: The area where new cells are produced for the growth of the nail plate; located under the cuticle and extends slightly onto the finger or toe.
4. Lunula: The crescent-shaped white area at the base of the nail plate, which is the visible portion of the matrix.
5. Cuticle: The thin layer of skin that overlaps the nail plate and protects the underlying tissue from infection.
6. Eponychium: The fold of skin that surrounds and covers the nail plate; also known as the "proximal nail fold."
7. Hyponychium: The area of skin between the free edge of the nail plate and the fingertip or toe tip.
8. Perionychiun: The skin surrounding the nail on all sides.

Understanding the anatomy and medical aspects of nails is essential for healthcare professionals, as various conditions can affect nail health, such as fungal infections, ingrown nails, or tumors.

Fibroblasts are specialized cells that play a critical role in the body's immune response and wound healing process. They are responsible for producing and maintaining the extracellular matrix (ECM), which is the non-cellular component present within all tissues and organs, providing structural support and biochemical signals for surrounding cells.

Fibroblasts produce various ECM proteins such as collagens, elastin, fibronectin, and laminins, forming a complex network of fibers that give tissues their strength and flexibility. They also help in the regulation of tissue homeostasis by controlling the turnover of ECM components through the process of remodeling.

In response to injury or infection, fibroblasts become activated and start to proliferate rapidly, migrating towards the site of damage. Here, they participate in the inflammatory response, releasing cytokines and chemokines that attract immune cells to the area. Additionally, they deposit new ECM components to help repair the damaged tissue and restore its functionality.

Dysregulation of fibroblast activity has been implicated in several pathological conditions, including fibrosis (excessive scarring), cancer (where they can contribute to tumor growth and progression), and autoimmune diseases (such as rheumatoid arthritis).

The dermis is the layer of skin located beneath the epidermis, which is the outermost layer of the skin. It is composed of connective tissue and provides structure and support to the skin. The dermis contains blood vessels, nerves, hair follicles, sweat glands, and oil glands. It is also responsible for the production of collagen and elastin, which give the skin its strength and flexibility. The dermis can be further divided into two layers: the papillary dermis, which is the upper layer and contains finger-like projections called papillae that extend upwards into the epidermis, and the reticular dermis, which is the lower layer and contains thicker collagen bundles. Together, the epidermis and dermis make up the true skin.

Autoantibodies are defined as antibodies that are produced by the immune system and target the body's own cells, tissues, or organs. These antibodies mistakenly identify certain proteins or molecules in the body as foreign invaders and attack them, leading to an autoimmune response. Autoantibodies can be found in various autoimmune diseases such as rheumatoid arthritis, lupus, and thyroiditis. The presence of autoantibodies can also be used as a diagnostic marker for certain conditions.

Antinuclear antibodies (ANA) are a type of autoantibody that target structures found in the nucleus of a cell. These antibodies are produced by the immune system and attack the body's own cells and tissues, leading to inflammation and damage. The presence of ANA is often used as a marker for certain autoimmune diseases, such as systemic lupus erythematosus (SLE), Sjogren's syndrome, rheumatoid arthritis, scleroderma, and polymyositis.

ANA can be detected through a blood test called the antinuclear antibody test. A positive result indicates the presence of ANA in the blood, but it does not necessarily mean that a person has an autoimmune disease. Further testing is usually needed to confirm a diagnosis and determine the specific type of autoantibodies present.

It's important to note that ANA can also be found in healthy individuals, particularly as they age. Therefore, the test results should be interpreted in conjunction with other clinical findings and symptoms.

Fibrosis is a pathological process characterized by the excessive accumulation and/or altered deposition of extracellular matrix components, particularly collagen, in various tissues and organs. This results in the formation of fibrous scar tissue that can impair organ function and structure. Fibrosis can occur as a result of chronic inflammation, tissue injury, or abnormal repair mechanisms, and it is a common feature of many diseases, including liver cirrhosis, lung fibrosis, heart failure, and kidney disease.

In medical terms, fibrosis is defined as:

"The process of producing scar tissue (consisting of collagen) in response to injury or chronic inflammation in normal connective tissue. This can lead to the thickening and stiffening of affected tissues and organs, impairing their function."

CREST syndrome is a subtype of a autoimmune connective tissue disorder called scleroderma (systemic sclerosis). The name "CREST" is an acronym that stands for the following five features:

* Calcinosis: The formation of calcium deposits in the skin and underlying tissues, which can cause painful ulcers.
* Raynaud's phenomenon: A condition in which the blood vessels in the fingers and toes constrict in response to cold or stress, causing the digits to turn white or blue and become numb or painful.
* Esophageal dysmotility: Difficulty swallowing due to weakened muscles in the esophagus.
* Sclerodactyly: Thickening and tightening of the skin on the fingers.
* Telangiectasias: Dilated blood vessels near the surface of the skin, causing red spots or lines.

It's important to note that not everyone with CREST syndrome will have all five of these features, and some people may have additional symptoms not included in the acronym. Additionally, CREST syndrome is a chronic condition that can cause a range of complications, including lung fibrosis, kidney problems, and digital ulcers. Treatment typically focuses on managing specific symptoms and slowing the progression of the disease.

Collagen Type I is the most abundant form of collagen in the human body, found in various connective tissues such as tendons, ligaments, skin, and bones. It is a structural protein that provides strength and integrity to these tissues. Collagen Type I is composed of three alpha chains, two alpha-1(I) chains, and one alpha-2(I) chain, arranged in a triple helix structure. This type of collagen is often used in medical research and clinical applications, such as tissue engineering and regenerative medicine, due to its excellent mechanical properties and biocompatibility.

Centromere Protein B (CENP-B) is a protein that plays a crucial role in the organization and function of centromeres, which are specialized regions of chromosomes where the spindle fibers attach during cell division. CENP-B is one of the proteins that make up the constitutive centromere-associated network (CCAN), which is a complex of proteins that forms the foundation of the kinetochore, the structure that connects the chromosome to the spindle fibers.

CENP-B has a unique ability to recognize and bind to specific DNA sequences within the centromere region called CENP-B boxes. This binding helps to establish and maintain the structural integrity of the centromere, ensuring that it functions correctly during cell division. Mutations in the CENP-B gene can lead to chromosomal instability and may contribute to the development of certain genetic disorders.

It's worth noting that while CENP-B is an important protein involved in centromere function, it is not present in all centromeres, and its absence does not necessarily mean that a centromere will be nonfunctional. Other proteins can compensate for the lack of CENP-B and help maintain centromere function.

Facial hemiatrophy, also known as Parry-Romberg syndrome, is a rare progressive condition characterized by the partial or complete atrophy (wasting) of the tissue on one side of the face. The atrophy typically involves the skin, fat, and muscle, but can also affect the bone and nerves.

The cause of facial hemiatrophy is not well understood, but it is believed to be a result of abnormalities in the blood vessels or nerves that supply the affected side of the face. The condition often begins in childhood or adolescence and can progress slowly over a period of several years.

In addition to the physical changes, people with facial hemiatrophy may also experience symptoms such as headaches, seizures, and eye problems. There is no cure for the condition, but various treatments such as cosmetic surgery, fillers, and muscle transfers can help improve the appearance of the affected side of the face.

Interstitial lung diseases (ILDs) are a group of disorders characterized by inflammation and scarring (fibrosis) in the interstitium, the tissue and space around the air sacs (alveoli) of the lungs. The interstitium is where the blood vessels that deliver oxygen to the lungs are located. ILDs can be caused by a variety of factors, including environmental exposures, medications, connective tissue diseases, and autoimmune disorders.

The scarring and inflammation in ILDs can make it difficult for the lungs to expand and contract normally, leading to symptoms such as shortness of breath, cough, and fatigue. The scarring can also make it harder for oxygen to move from the air sacs into the bloodstream.

There are many different types of ILDs, including:

* Idiopathic pulmonary fibrosis (IPF): a type of ILD that is caused by unknown factors and tends to progress rapidly
* Hypersensitivity pneumonitis: an ILD that is caused by an allergic reaction to inhaled substances, such as mold or bird droppings
* Connective tissue diseases: ILDs can be a complication of conditions such as rheumatoid arthritis and scleroderma
* Sarcoidosis: an inflammatory disorder that can affect multiple organs, including the lungs
* Asbestosis: an ILD caused by exposure to asbestos fibers

Treatment for ILDs depends on the specific type of disease and its underlying cause. Some treatments may include corticosteroids, immunosuppressive medications, and oxygen therapy. In some cases, a lung transplant may be necessary.

Connective tissue diseases (CTDs) are a group of disorders that involve the abnormal production and accumulation of abnormal connective tissues in various parts of the body. Connective tissues are the structural materials that support and bind other tissues and organs together. They include tendons, ligaments, cartilage, fat, and the material that fills the spaces between cells, called the extracellular matrix.

Connective tissue diseases can affect many different systems in the body, including the skin, joints, muscles, lungs, kidneys, gastrointestinal tract, and blood vessels. Some CTDs are autoimmune disorders, meaning that the immune system mistakenly attacks healthy connective tissues. Others may be caused by genetic mutations or environmental factors.

Some examples of connective tissue diseases include:

* Systemic lupus erythematosus (SLE)
* Rheumatoid arthritis (RA)
* Scleroderma
* Dermatomyositis/Polymyositis
* Mixed Connective Tissue Disease (MCTD)
* Sjogren's syndrome
* Ehlers-Danlos syndrome
* Marfan syndrome
* Osteogenesis imperfecta

The specific symptoms and treatment of connective tissue diseases vary depending on the type and severity of the condition. Treatment may include medications to reduce inflammation, suppress the immune system, or manage pain. In some cases, surgery may be necessary to repair or replace damaged tissues or organs.

Bleomycin is a type of chemotherapeutic agent used to treat various types of cancer, including squamous cell carcinoma, testicular cancer, and lymphomas. It works by causing DNA damage in rapidly dividing cells, which can inhibit the growth and proliferation of cancer cells.

Bleomycin is an antibiotic derived from Streptomyces verticillus and is often administered intravenously or intramuscularly. While it can be effective in treating certain types of cancer, it can also have serious side effects, including lung toxicity, which can lead to pulmonary fibrosis and respiratory failure. Therefore, bleomycin should only be used under the close supervision of a healthcare professional who is experienced in administering chemotherapy drugs.

I'm sorry for any confusion, but "hobbies" is not a term that has a medical definition. Hobbies generally refer to activities or interests that are pursued outside of one's professional or obligatory duties, purely for pleasure and relaxation. They can include a wide range of activities such as painting, hiking, playing a musical instrument, gardening, or bird watching, among many others. If you have any questions related to medical topics, I'd be happy to try to help answer those!

Transforming Growth Factor-beta (TGF-β) is a type of cytokine, which is a cell signaling protein involved in the regulation of various cellular processes, including cell growth, differentiation, and apoptosis (programmed cell death). TGF-β plays a critical role in embryonic development, tissue homeostasis, and wound healing. It also has been implicated in several pathological conditions such as fibrosis, cancer, and autoimmune diseases.

TGF-β exists in multiple isoforms (TGF-β1, TGF-β2, and TGF-β3) that are produced by many different cell types, including immune cells, epithelial cells, and fibroblasts. The protein is synthesized as a precursor molecule, which is cleaved to release the active TGF-β peptide. Once activated, TGF-β binds to its receptors on the cell surface, leading to the activation of intracellular signaling pathways that regulate gene expression and cell behavior.

In summary, Transforming Growth Factor-beta (TGF-β) is a multifunctional cytokine involved in various cellular processes, including cell growth, differentiation, apoptosis, embryonic development, tissue homeostasis, and wound healing. It has been implicated in several pathological conditions such as fibrosis, cancer, and autoimmune diseases.

A skin ulcer is a defined as a loss of continuity or disruption of the skin surface, often accompanied by inflammation and/or infection. These lesions can result from various causes including pressure, venous or arterial insufficiency, diabetes, and chronic dermatological conditions. Skin ulcers are typically characterized by their appearance, depth, location, and underlying cause. Common types of skin ulcers include pressure ulcers (also known as bedsores), venous leg ulcers, arterial ulcers, and diabetic foot ulcers. Proper evaluation, wound care, management of underlying conditions, and prevention strategies are crucial in the treatment of skin ulcers to promote healing and prevent complications.

Connective Tissue Growth Factor (CTGF) is a cysteine-rich peptide growth factor that belongs to the CCN family of proteins. It plays an important role in various biological processes, including cell adhesion, migration, proliferation, and extracellular matrix production. CTGF is involved in wound healing, tissue repair, and fibrosis, as well as in the pathogenesis of several diseases such as cancer, diabetic nephropathy, and systemic sclerosis. It is expressed in response to various stimuli, including growth factors, cytokines, and mechanical stress. CTGF interacts with a variety of signaling molecules and integrins to regulate cellular responses and tissue homeostasis.

Collagen is the most abundant protein in the human body, and it is a major component of connective tissues such as tendons, ligaments, skin, and bones. Collagen provides structure and strength to these tissues and helps them to withstand stretching and tension. It is made up of long chains of amino acids, primarily glycine, proline, and hydroxyproline, which are arranged in a triple helix structure. There are at least 16 different types of collagen found in the body, each with slightly different structures and functions. Collagen is important for maintaining the integrity and health of tissues throughout the body, and it has been studied for its potential therapeutic uses in various medical conditions.

Pulmonary fibrosis is a specific type of lung disease that results from the thickening and scarring of the lung tissues, particularly those in the alveoli (air sacs) and interstitium (the space around the air sacs). This scarring makes it harder for the lungs to properly expand and transfer oxygen into the bloodstream, leading to symptoms such as shortness of breath, coughing, fatigue, and eventually respiratory failure. The exact cause of pulmonary fibrosis can vary, with some cases being idiopathic (without a known cause) or related to environmental factors, medications, medical conditions, or genetic predisposition.

Autoimmune diseases are a group of disorders in which the immune system, which normally protects the body from foreign invaders like bacteria and viruses, mistakenly attacks the body's own cells and tissues. This results in inflammation and damage to various organs and tissues in the body.

In autoimmune diseases, the body produces autoantibodies that target its own proteins or cell receptors, leading to their destruction or malfunction. The exact cause of autoimmune diseases is not fully understood, but it is believed that a combination of genetic and environmental factors contribute to their development.

There are over 80 different types of autoimmune diseases, including rheumatoid arthritis, lupus, multiple sclerosis, type 1 diabetes, Hashimoto's thyroiditis, Graves' disease, psoriasis, and inflammatory bowel disease. Symptoms can vary widely depending on the specific autoimmune disease and the organs or tissues affected. Treatment typically involves managing symptoms and suppressing the immune system to prevent further damage.

Pulmonary hypertension is a medical condition characterized by increased blood pressure in the pulmonary arteries, which are the blood vessels that carry blood from the right side of the heart to the lungs. This results in higher than normal pressures in the pulmonary circulation and can lead to various symptoms and complications.

Pulmonary hypertension is typically defined as a mean pulmonary artery pressure (mPAP) greater than or equal to 25 mmHg at rest, as measured by right heart catheterization. The World Health Organization (WHO) classifies pulmonary hypertension into five groups based on the underlying cause:

1. Pulmonary arterial hypertension (PAH): This group includes idiopathic PAH, heritable PAH, drug-induced PAH, and associated PAH due to conditions such as connective tissue diseases, HIV infection, portal hypertension, congenital heart disease, and schistosomiasis.
2. Pulmonary hypertension due to left heart disease: This group includes conditions that cause elevated left atrial pressure, such as left ventricular systolic or diastolic dysfunction, valvular heart disease, and congenital cardiovascular shunts.
3. Pulmonary hypertension due to lung diseases and/or hypoxia: This group includes chronic obstructive pulmonary disease (COPD), interstitial lung disease, sleep-disordered breathing, alveolar hypoventilation disorders, and high altitude exposure.
4. Chronic thromboembolic pulmonary hypertension (CTEPH): This group includes persistent obstruction of the pulmonary arteries due to organized thrombi or emboli.
5. Pulmonary hypertension with unclear and/or multifactorial mechanisms: This group includes hematologic disorders, systemic disorders, metabolic disorders, and other conditions that can cause pulmonary hypertension but do not fit into the previous groups.

Symptoms of pulmonary hypertension may include shortness of breath, fatigue, chest pain, lightheadedness, and syncope (fainting). Diagnosis typically involves a combination of medical history, physical examination, imaging studies, and invasive testing such as right heart catheterization. Treatment depends on the underlying cause but may include medications, oxygen therapy, pulmonary rehabilitation, and, in some cases, surgical intervention.

Rheumatic diseases are a group of disorders that cause pain, stiffness, and swelling in the joints, muscles, tendons, ligaments, or bones. They include conditions such as rheumatoid arthritis, osteoarthritis, systemic lupus erythematosus (SLE), gout, ankylosing spondylitis, psoriatic arthritis, and many others. These diseases can also affect other body systems including the skin, eyes, lungs, heart, kidneys, and nervous system. Rheumatic diseases are often chronic and may be progressive, meaning they can worsen over time. They can cause significant pain, disability, and reduced quality of life if not properly diagnosed and managed. The exact causes of rheumatic diseases are not fully understood, but genetics, environmental factors, and immune system dysfunction are believed to play a role in their development.

PUVA therapy is a type of treatment that uses both medication and light to treat certain skin conditions, such as psoriasis, eczema, and cutaneous T-cell lymphoma. The name "PUVA" stands for Psoralen + UVA, which refers to the two main components of the therapy:

1. Psoralen: This is a medication that makes the skin more sensitive to light. It can be taken orally or applied directly to the skin in the form of a cream or bath.
2. UVA: This stands for Ultraviolet A, which is a type of light that is part of the natural sunlight spectrum. In PUVA therapy, the skin is exposed to a controlled dose of UVA light in a special booth or room.

When psoralen is introduced into the body, it absorbs into the skin and makes it more sensitive to UVA light. When the skin is then exposed to UVA light, it triggers a chemical reaction that slows down the growth of affected skin cells. This helps to reduce inflammation, scaling, and other symptoms associated with the skin condition being treated.

It's important to note that PUVA therapy can have side effects, including sunburn, itching, redness, and an increased risk of skin cancer over time. As such, it is typically used as a second-line treatment when other therapies have not been effective, and it is closely monitored by a healthcare professional to ensure its safe and effective use.

Esophageal diseases refer to a range of medical conditions that affect the esophagus, which is the muscular tube that connects the throat to the stomach. Here are some common esophageal diseases with their brief definitions:

1. Gastroesophageal reflux disease (GERD): A chronic condition in which stomach acid or bile flows back into the esophagus, causing symptoms such as heartburn, chest pain, and difficulty swallowing.
2. Esophagitis: Inflammation of the esophageal lining, often caused by GERD, infection, or medication.
3. Esophageal stricture: Narrowing of the esophagus due to scarring or inflammation, which can make swallowing difficult.
4. Esophageal cancer: Cancer that forms in the tissues of the esophagus, often as a result of long-term GERD or smoking.
5. Esophageal motility disorders: Disorders that affect the normal movement and function of the esophagus, such as achalasia, diffuse spasm, and nutcracker esophagus.
6. Barrett's esophagus: A condition in which the lining of the lower esophagus changes, increasing the risk of esophageal cancer.
7. Esophageal diverticula: Small pouches that form in the esophageal wall, often causing difficulty swallowing or regurgitation.
8. Eosinophilic esophagitis (EoE): A chronic immune-mediated disorder characterized by inflammation of the esophagus due to an allergic reaction.

These are some of the common esophageal diseases, and their diagnosis and treatment may vary depending on the severity and underlying cause of the condition.

Autoantigens are substances that are typically found in an individual's own body, but can stimulate an immune response because they are recognized as foreign by the body's own immune system. In autoimmune diseases, the immune system mistakenly attacks and damages healthy tissues and organs because it recognizes some of their components as autoantigens. These autoantigens can be proteins, DNA, or other molecules that are normally present in the body but have become altered or exposed due to various factors such as infection, genetics, or environmental triggers. The immune system then produces antibodies and activates immune cells to attack these autoantigens, leading to tissue damage and inflammation.

Silicones are not a medical term, but they are commonly used in the medical field, particularly in medical devices and healthcare products. Silicones are synthetic polymers made up of repeating units of siloxane, which is a chain of alternating silicon and oxygen atoms. They can exist in various forms such as oils, gels, rubbers, and resins.

In the medical context, silicones are often used for their unique properties, including:

1. Biocompatibility - Silicones have a low risk of causing an adverse reaction when they come into contact with living tissue.
2. Inertness - They do not react chemically with other substances, making them suitable for use in medical devices that need to remain stable over time.
3. Temperature resistance - Silicones can maintain their flexibility and elasticity even under extreme temperature conditions.
4. Gas permeability - Some silicone materials allow gases like oxygen and water vapor to pass through, which is useful in applications where maintaining a moist environment is essential.
5. Durability - Silicones have excellent resistance to aging, weathering, and environmental factors, ensuring long-lasting performance.

Examples of medical applications for silicones include:

1. Breast implants
2. Contact lenses
3. Catheters
4. Artificial joints and tendons
5. Bandages and wound dressings
6. Drug delivery systems
7. Medical adhesives
8. Infant care products (nipples, pacifiers)

Mixed Connective Tissue Disease (MCTD) is a rare overlapping condition of the connective tissues, characterized by the presence of specific autoantibodies against a protein called "U1-snRNP" or "U1-small nuclear ribonucleoprotein." This disorder has features of various connective tissue diseases such as systemic lupus erythematosus (SLE), scleroderma, polymyositis, and rheumatoid arthritis. Symptoms may include swollen hands, joint pain and swelling, muscle weakness, skin thickening, lung involvement, and Raynaud's phenomenon. The exact cause of MCTD is unknown, but it is believed to involve both genetic and environmental factors leading to an autoimmune response. Early diagnosis and treatment are essential for better disease management and preventing severe complications.

Smad3 protein is a transcription factor that plays a crucial role in the TGF-β (transforming growth factor-beta) signaling pathway. When TGF-β binds to its receptor, it activates Smad3 through phosphorylation. Activated Smad3 then forms a complex with other Smad proteins and translocates into the nucleus where it regulates the transcription of target genes involved in various cellular processes such as proliferation, differentiation, apoptosis, and migration.

Mutations in the SMAD3 gene or dysregulation of the TGF-β/Smad3 signaling pathway have been implicated in several human diseases, including fibrotic disorders, cancer, and Marfan syndrome. Therefore, Smad3 protein is an important target for therapeutic interventions in these conditions.

Microstomia is a medical term that refers to an abnormally small or narrow opening of the mouth. This condition can result from various causes, including congenital disorders, surgical procedures, or neuromuscular diseases. Microstomia can lead to difficulties with speaking, eating, oral hygiene, and dental care. Treatment options may include physical therapy, surgery, or the use of specialized medical devices to help widen the mouth opening.

Eye manifestations refer to any changes or abnormalities in the eye that can be observed or detected. These manifestations can be related to various medical conditions, diseases, or disorders affecting the eye or other parts of the body. They can include structural changes, such as swelling or bulging of the eye, as well as functional changes, such as impaired vision or sensitivity to light. Examples of eye manifestations include cataracts, glaucoma, diabetic retinopathy, macular degeneration, and uveitis.

DNA topoisomerases are enzymes that modify the topological structure of DNA by regulating the number of twists or supercoils in the double helix. There are two main types of DNA topoisomerases: type I and type II.

Type I DNA topoisomerases function by cutting one strand of the DNA duplex, allowing the uncut strand to rotate around the break, and then resealing the break. This process can relieve both positive and negative supercoiling in DNA, as well as introduce single-stranded breaks into the DNA molecule.

Type I topoisomerases are further divided into three subtypes: type IA, type IB, and type IC. These subtypes differ in their mechanism of action and the structure of the active site tyrosine residue that makes the transient break in the DNA strand.

Overall, DNA topoisomerases play a crucial role in many cellular processes involving DNA, including replication, transcription, recombination, and chromosome segregation. Dysregulation of these enzymes has been implicated in various human diseases, including cancer and genetic disorders.

Skin diseases, also known as dermatological conditions, refer to any medical condition that affects the skin, which is the largest organ of the human body. These diseases can affect the skin's function, appearance, or overall health. They can be caused by various factors, including genetics, infections, allergies, environmental factors, and aging.

Skin diseases can present in many different forms, such as rashes, blisters, sores, discolorations, growths, or changes in texture. Some common examples of skin diseases include acne, eczema, psoriasis, dermatitis, fungal infections, viral infections, bacterial infections, and skin cancer.

The symptoms and severity of skin diseases can vary widely depending on the specific condition and individual factors. Some skin diseases are mild and can be treated with over-the-counter medications or topical creams, while others may require more intensive treatments such as prescription medications, light therapy, or even surgery.

It is important to seek medical attention if you experience any unusual or persistent changes in your skin, as some skin diseases can be serious or indicative of other underlying health conditions. A dermatologist is a medical doctor who specializes in the diagnosis and treatment of skin diseases.

A centromere is a specialized region found on chromosomes that plays a crucial role in the separation of replicated chromosomes during cell division. It is the point where the sister chromatids (the two copies of a chromosome formed during DNA replication) are joined together. The centromere contains highly repeated DNA sequences and proteins that form a complex structure known as the kinetochore, which serves as an attachment site for microtubules of the mitotic spindle during cell division.

During mitosis or meiosis, the kinetochore facilitates the movement of chromosomes by interacting with the microtubules, allowing for the accurate distribution of genetic material to the daughter cells. Centromeres can vary in their position and structure among different species, ranging from being located near the middle of the chromosome (metacentric) to being positioned closer to one end (acrocentric). The precise location and characteristics of centromeres are essential for proper chromosome segregation and maintenance of genomic stability.

Vesiculobullous skin diseases are a group of disorders characterized by the formation of blisters (vesicles) and bullae (larger blisters) on the skin. These blisters form when there is a separation between the epidermis (outer layer of the skin) and the dermis (layer beneath the epidermis) due to damage in the area where they join, known as the dermo-epidermal junction.

There are several types of vesiculobullous diseases, each with its own specific causes and symptoms. Some of the most common types include:

1. Pemphigus vulgaris: an autoimmune disorder where the immune system mistakenly attacks proteins that help to hold the skin together, causing blisters to form.
2. Bullous pemphigoid: another autoimmune disorder, but in this case, the immune system attacks a different set of proteins, leading to large blisters and inflammation.
3. Dermatitis herpetiformis: a skin condition associated with celiac disease, where gluten ingestion triggers an immune response that leads to the formation of itchy blisters.
4. Pemphigoid gestationis: a rare autoimmune disorder that occurs during pregnancy and causes blisters on the abdomen and other parts of the body.
5. Epidermolysis bullosa: a group of inherited disorders where there is a fragile skin structure, leading to blistering and wound formation after minor trauma or friction.

Treatment for vesiculobullous diseases depends on the specific diagnosis and may include topical or systemic medications, such as corticosteroids, immunosuppressants, or antibiotics, as well as wound care and prevention of infection.

Pulmonary diffusing capacity, also known as pulmonary diffusion capacity, is a measure of the ability of the lungs to transfer gas from the alveoli to the bloodstream. It is often used to assess the severity of lung diseases such as chronic obstructive pulmonary disease (COPD) and pulmonary fibrosis.

The most common measurement of pulmonary diffusing capacity is the diffusing capacity for carbon monoxide (DLCO), which reflects the transfer of carbon monoxide from the alveoli to the red blood cells in the capillaries. The DLCO is measured during a spirometry test, which involves breathing in a small amount of carbon monoxide and then measuring how much of it is exhaled.

A reduced DLCO may indicate a problem with the lung's ability to transfer oxygen to the blood, which can be caused by a variety of factors including damage to the alveoli or capillaries, thickening of the alveolar membrane, or a decrease in the surface area available for gas exchange.

It is important to note that other factors such as hemoglobin concentration, carboxyhemoglobin level, and lung volume can also affect the DLCO value, so these should be taken into account when interpreting the results of a diffusing capacity test.

The Ulnar Artery is a major blood vessel that supplies the forearm, hand, and fingers with oxygenated blood. It originates from the brachial artery in the upper arm and travels down the medial (towards the body's midline) side of the forearm, passing through the Guyon's canal at the wrist before branching out to supply the hand and fingers.

The ulnar artery provides blood to the palmar aspect of the hand and the ulnar side of the little finger and half of the ring finger. It also contributes to the formation of the deep palmar arch, which supplies blood to the deep structures of the hand. The ulnar artery is an important structure in the circulatory system, providing critical blood flow to the upper limb.

Penicillamine is a medication that belongs to a class of drugs called chelating agents. It works by binding to heavy metals in the body, such as lead, mercury, or copper, and forming a compound that can be excreted in the urine. This helps to remove these harmful substances from the body.

Penicillamine is also used to treat certain medical conditions, such as rheumatoid arthritis, Wilson's disease (a genetic disorder that causes copper accumulation in the body), and cystinuria (a genetic disorder that causes an amino acid called cystine to accumulate in the kidneys and form stones).

It is important to note that penicillamine can have serious side effects, including kidney damage, so it should be used under the close supervision of a healthcare provider.

"Cells, cultured" is a medical term that refers to cells that have been removed from an organism and grown in controlled laboratory conditions outside of the body. This process is called cell culture and it allows scientists to study cells in a more controlled and accessible environment than they would have inside the body. Cultured cells can be derived from a variety of sources, including tissues, organs, or fluids from humans, animals, or cell lines that have been previously established in the laboratory.

Cell culture involves several steps, including isolation of the cells from the tissue, purification and characterization of the cells, and maintenance of the cells in appropriate growth conditions. The cells are typically grown in specialized media that contain nutrients, growth factors, and other components necessary for their survival and proliferation. Cultured cells can be used for a variety of purposes, including basic research, drug development and testing, and production of biological products such as vaccines and gene therapies.

It is important to note that cultured cells may behave differently than they do in the body, and results obtained from cell culture studies may not always translate directly to human physiology or disease. Therefore, it is essential to validate findings from cell culture experiments using additional models and ultimately in clinical trials involving human subjects.

A forehead, in medical terms, refers to the portion of the human skull that lies immediately above the eyes and serves as an attachment site for the frontal bone. It is a common area for the examination of various clinical signs, such as assessing the level of consciousness (by checking if the patient's eyebrows or eyelids twitch in response to a light touch) or looking for signs of increased intracranial pressure (such as bulging fontanelles in infants). Additionally, the forehead is often used as a site for non-invasive procedures like Botox injections.

Capillaries are the smallest blood vessels in the body, with diameters that range from 5 to 10 micrometers. They form a network of tiny tubes that connect the arterioles (small branches of arteries) and venules (small branches of veins), allowing for the exchange of oxygen, carbon dioxide, nutrients, and waste products between the blood and the surrounding tissues.

Capillaries are composed of a single layer of endothelial cells that surround a hollow lumen through which blood flows. The walls of capillaries are extremely thin, allowing for easy diffusion of molecules between the blood and the surrounding tissue. This is essential for maintaining the health and function of all body tissues.

Capillaries can be classified into three types based on their structure and function: continuous, fenestrated, and sinusoidal. Continuous capillaries have a continuous layer of endothelial cells with tight junctions that restrict the passage of large molecules. Fenestrated capillaries have small pores or "fenestrae" in the endothelial cell walls that allow for the passage of larger molecules, such as proteins and lipids. Sinusoidal capillaries are found in organs with high metabolic activity, such as the liver and spleen, and have large, irregular spaces between the endothelial cells that allow for the exchange of even larger molecules.

Overall, capillaries play a critical role in maintaining the health and function of all body tissues by allowing for the exchange of nutrients, oxygen, and waste products between the blood and surrounding tissues.

Collagen diseases, also known as collagen disorders or connective tissue diseases, refer to a group of medical conditions that affect the body's connective tissues. These tissues provide support and structure for various organs and systems in the body, including the skin, joints, muscles, and blood vessels.

Collagen is a major component of connective tissues, and it plays a crucial role in maintaining their strength and elasticity. In collagen diseases, the body's immune system mistakenly attacks healthy collagen, leading to inflammation, pain, and damage to the affected tissues.

There are several types of collagen diseases, including:

1. Systemic Lupus Erythematosus (SLE): This is a chronic autoimmune disease that can affect various organs and systems in the body, including the skin, joints, kidneys, heart, and lungs.
2. Rheumatoid Arthritis (RA): This is a chronic inflammatory disease that primarily affects the joints, causing pain, swelling, and stiffness.
3. Scleroderma: This is a rare autoimmune disorder that causes thickening and hardening of the skin and connective tissues, leading to restricted movement and organ damage.
4. Dermatomyositis: This is an inflammatory muscle disease that can also affect the skin, causing rashes and weakness.
5. Mixed Connective Tissue Disease (MCTD): This is a rare autoimmune disorder that combines symptoms of several collagen diseases, including SLE, RA, scleroderma, and dermatomyositis.

The exact cause of collagen diseases is not fully understood, but they are believed to be related to genetic, environmental, and hormonal factors. Treatment typically involves a combination of medications, lifestyle changes, and physical therapy to manage symptoms and prevent complications.

Breast implants are medical devices that are inserted into the breast to enhance their size, shape, or fullness. They can also be used for breast reconstruction after a mastectomy or other medical treatments. Breast implants typically consist of a silicone shell filled with either saline (sterile saltwater) or silicone gel.

There are two main types of breast implants:

1. Saline-filled implants: These implants have a silicone outer shell that is filled with sterile saline solution after the implant has been inserted into the breast. This allows for some adjustment in the size and shape of the implant after surgery.
2. Silicone gel-filled implants: These implants have a silicone outer shell that is pre-filled with a cohesive silicone gel. The gel is designed to feel more like natural breast tissue than saline implants.

Breast implants come in various sizes, shapes, and textures, and the choice of implant will depend on several factors, including the patient's body type, desired outcome, and personal preference. It is important for patients considering breast implants to discuss their options with a qualified plastic surgeon who can help them make an informed decision based on their individual needs and goals.

Scalp dermatoses refer to various skin conditions that affect the scalp. These can include inflammatory conditions such as seborrheic dermatitis (dandruff, cradle cap), psoriasis, atopic dermatitis (eczema), and lichen planus; infectious processes like bacterial folliculitis, tinea capitis (ringworm of the scalp), and viral infections; as well as autoimmune conditions such as alopecia areata. Symptoms can range from mild scaling and itching to severe redness, pain, and hair loss. The specific diagnosis and treatment of scalp dermatoses depend on the underlying cause.

Systemic Lupus Erythematosus (SLE) is a complex autoimmune disease that can affect almost any organ or system in the body. In SLE, the immune system produces an exaggerated response, leading to the production of autoantibodies that attack the body's own cells and tissues, causing inflammation and damage. The symptoms and severity of SLE can vary widely from person to person, but common features include fatigue, joint pain, skin rashes (particularly a "butterfly" rash across the nose and cheeks), fever, hair loss, and sensitivity to sunlight.

Systemic lupus erythematosus can also affect the kidneys, heart, lungs, brain, blood vessels, and other organs, leading to a wide range of symptoms such as kidney dysfunction, chest pain, shortness of breath, seizures, and anemia. The exact cause of SLE is not fully understood, but it is believed to involve a combination of genetic, environmental, and hormonal factors. Treatment typically involves medications to suppress the immune system and manage symptoms, and may require long-term management by a team of healthcare professionals.

I'm sorry for any confusion, but "South Australia" is not a medical term or concept. It's actually the name of a region, specifically the Australian state of South Australia. If you have any questions related to medical terminology or health-related topics, I'd be happy to try and help with those!

Gastrointestinal diseases refer to a group of conditions that affect the gastrointestinal (GI) tract, which includes the organs from the mouth to the anus, responsible for food digestion, absorption, and elimination of waste. These diseases can affect any part of the GI tract, causing various symptoms such as abdominal pain, bloating, diarrhea, constipation, nausea, vomiting, and weight loss.

Common gastrointestinal diseases include:

1. Gastroesophageal reflux disease (GERD) - a condition where stomach acid flows back into the esophagus, causing heartburn and other symptoms.
2. Peptic ulcers - sores that develop in the lining of the stomach or duodenum, often caused by bacterial infection or long-term use of nonsteroidal anti-inflammatory drugs (NSAIDs).
3. Inflammatory bowel disease (IBD) - a group of chronic inflammatory conditions of the intestine, including Crohn's disease and ulcerative colitis.
4. Irritable bowel syndrome (IBS) - a functional gastrointestinal disorder characterized by abdominal pain, bloating, and altered bowel habits.
5. Celiac disease - an autoimmune disorder where the ingestion of gluten leads to damage in the small intestine.
6. Diverticular disease - a condition that affects the colon, causing diverticula (small pouches) to form and potentially become inflamed or infected.
7. Constipation - a common gastrointestinal symptom characterized by infrequent bowel movements, hard stools, and difficulty passing stools.
8. Diarrhea - a common gastrointestinal symptom characterized by loose, watery stools and frequent bowel movements.
9. Food intolerances and allergies - adverse reactions to specific foods or food components that can cause various gastrointestinal symptoms.
10. Gastrointestinal infections - caused by bacteria, viruses, parasites, or fungi that can lead to a range of symptoms, including diarrhea, vomiting, and abdominal pain.

Smad7 protein is a intracellular signaling molecule that plays a role in negative regulation of the transforming growth factor-beta (TGF-β) superfamily of cytokines. It is a member of the Smad family, which are proteins that transduce signals from the cell membrane to the nucleus in response to TGF-β ligands binding to their receptors.

Smad7 functions as an inhibitory Smad by blocking the formation of active Smad complexes and targeting the activated type I TGF-β receptor for degradation, thus preventing the activation of TGF-β signaling pathways. It also interacts with other signaling molecules, such as tumor necrosis factor-associated factor 6 (TRAF6) and transforming growth factor-beta-activated kinase 1 (TAK1), to inhibit their activity and downregulate TGF-β signaling.

Abnormal regulation of Smad7 protein has been implicated in various human diseases, including fibrosis, cancer, and autoimmune disorders.

Sclerosis is a medical term that refers to the abnormal hardening or scarring of body tissues, particularly in the context of various degenerative diseases affecting the nervous system. The term "sclerosis" comes from the Greek word "skleros," which means hard. In these conditions, the normally flexible and adaptable nerve cells or their protective coverings (myelin sheath) become rigid and inflexible due to the buildup of scar tissue or abnormal protein deposits.

There are several types of sclerosis, but one of the most well-known is multiple sclerosis (MS). In MS, the immune system mistakenly attacks the myelin sheath surrounding nerve fibers in the brain and spinal cord, leading to scarring and damage that disrupts communication between the brain and the rest of the body. This results in a wide range of symptoms, such as muscle weakness, numbness, vision problems, balance issues, and cognitive impairment.

Other conditions that involve sclerosis include:

1. Amyotrophic lateral sclerosis (ALS): Also known as Lou Gehrig's disease, ALS is a progressive neurodegenerative disorder affecting motor neurons in the brain and spinal cord, leading to muscle weakness, stiffness, and atrophy.
2. Systemic sclerosis: A rare autoimmune connective tissue disorder characterized by thickening and hardening of the skin and internal organs due to excessive collagen deposition.
3. Plaque psoriasis: A chronic inflammatory skin condition marked by red, scaly patches (plaques) resulting from rapid turnover and accumulation of skin cells.
4. Adhesive capsulitis: Also known as frozen shoulder, this condition involves stiffening and thickening of the shoulder joint's capsule due to scarring or inflammation, leading to limited mobility and pain.

Lung diseases refer to a broad category of disorders that affect the lungs and other structures within the respiratory system. These diseases can impair lung function, leading to symptoms such as coughing, shortness of breath, chest pain, and wheezing. They can be categorized into several types based on the underlying cause and nature of the disease process. Some common examples include:

1. Obstructive lung diseases: These are characterized by narrowing or blockage of the airways, making it difficult to breathe out. Examples include chronic obstructive pulmonary disease (COPD), asthma, bronchiectasis, and cystic fibrosis.
2. Restrictive lung diseases: These involve stiffening or scarring of the lungs, which reduces their ability to expand and take in air. Examples include idiopathic pulmonary fibrosis, sarcoidosis, and asbestosis.
3. Infectious lung diseases: These are caused by bacteria, viruses, fungi, or parasites that infect the lungs. Examples include pneumonia, tuberculosis, and influenza.
4. Vascular lung diseases: These affect the blood vessels in the lungs, impairing oxygen exchange. Examples include pulmonary embolism, pulmonary hypertension, and chronic thromboembolic pulmonary hypertension (CTEPH).
5. Neoplastic lung diseases: These involve abnormal growth of cells within the lungs, leading to cancer. Examples include small cell lung cancer, non-small cell lung cancer, and mesothelioma.
6. Other lung diseases: These include interstitial lung diseases, pleural effusions, and rare disorders such as pulmonary alveolar proteinosis and lymphangioleiomyomatosis (LAM).

It is important to note that this list is not exhaustive, and there are many other conditions that can affect the lungs. Proper diagnosis and treatment of lung diseases require consultation with a healthcare professional, such as a pulmonologist or respiratory therapist.

Dermatomyositis is a medical condition characterized by inflammation and weakness in the muscles and skin. It is a type of inflammatory myopathy, which means that it causes muscle inflammation and damage. Dermatomyositis is often associated with a distinctive rash that affects the skin around the eyes, nose, mouth, fingers, and toes.

The symptoms of dermatomyositis can include:

* Progressive muscle weakness, particularly in the hips, thighs, shoulders, and neck
* Fatigue
* Difficulty swallowing or speaking
* Skin rash, which may be pink or purple and is often accompanied by itching
* Muscle pain and tenderness
* Joint pain and swelling
* Raynaud's phenomenon, a condition that affects blood flow to the fingers and toes

The exact cause of dermatomyositis is not known, but it is believed to be related to an autoimmune response in which the body's immune system mistakenly attacks healthy tissue. Treatment for dermatomyositis typically involves medications to reduce inflammation and suppress the immune system, as well as physical therapy to help maintain muscle strength and function.

Ultraviolet (UV) therapy, also known as phototherapy, is a medical treatment that uses ultraviolet light to treat various skin conditions. The UV light can be delivered through natural sunlight or artificial sources, such as specialized lamps or lasers.

In medical settings, controlled doses of UV light are used to target specific areas of the skin. The most common type of UV therapy is narrowband UVB (NB-UVB) phototherapy, which uses a specific wavelength of UVB light to treat conditions such as psoriasis, eczema, vitiligo, and dermatitis.

The goal of UV therapy is to reduce inflammation, slow skin cell growth, and improve the overall appearance of the skin. It is important to note that while UV therapy can be effective in treating certain skin conditions, it also carries risks such as skin aging and an increased risk of skin cancer. Therefore, it should only be administered under the supervision of a qualified healthcare professional.

Respiratory Function Tests (RFTs) are a group of medical tests that measure how well your lungs take in and exhale air, and how well they transfer oxygen and carbon dioxide into and out of your blood. They can help diagnose certain lung disorders, measure the severity of lung disease, and monitor response to treatment.

RFTs include several types of tests, such as:

1. Spirometry: This test measures how much air you can exhale and how quickly you can do it. It's often used to diagnose and monitor conditions like asthma, chronic obstructive pulmonary disease (COPD), and other lung diseases.
2. Lung volume testing: This test measures the total amount of air in your lungs. It can help diagnose restrictive lung diseases, such as pulmonary fibrosis or sarcoidosis.
3. Diffusion capacity testing: This test measures how well oxygen moves from your lungs into your bloodstream. It's often used to diagnose and monitor conditions like pulmonary fibrosis, interstitial lung disease, and other lung diseases that affect the ability of the lungs to transfer oxygen to the blood.
4. Bronchoprovocation testing: This test involves inhaling a substance that can cause your airways to narrow, such as methacholine or histamine. It's often used to diagnose and monitor asthma.
5. Exercise stress testing: This test measures how well your lungs and heart work together during exercise. It's often used to diagnose lung or heart disease.

Overall, Respiratory Function Tests are an important tool for diagnosing and managing a wide range of lung conditions.

Pericardial effusion is an abnormal accumulation of fluid in the pericardial space, which is the potential space between the two layers of the pericardium - the fibrous and serous layers. The pericardium is a sac that surrounds the heart to provide protection and lubrication for the heart's movement during each heartbeat. Normally, there is only a small amount of fluid (5-15 mL) in this space to ensure smooth motion of the heart. However, when an excessive amount of fluid accumulates, it can cause increased pressure on the heart, leading to various complications such as decreased cardiac output and even cardiac tamponade, a life-threatening condition that requires immediate medical attention.

Pericardial effusion may result from several causes, including infections (viral, bacterial, or fungal), inflammatory conditions (such as rheumatoid arthritis, lupus, or cancer), trauma, heart surgery, kidney failure, or iatrogenic causes. The symptoms of pericardial effusion can vary depending on the rate and amount of fluid accumulation. Slowly developing effusions may not cause any symptoms, while rapid accumulations can lead to chest pain, shortness of breath, cough, palpitations, or even hypotension (low blood pressure). Diagnosis is usually confirmed through imaging techniques such as echocardiography, CT scan, or MRI. Treatment depends on the underlying cause and severity of the effusion, ranging from close monitoring to drainage procedures or medications to address the root cause.

... (Localized Scleroderma. Nicole M. Fett, MD. JAMA Dermatol. 2013;149(9):1124. doi:10.1001/jamadermatol.2013.5079. ... Peterson LS, Nelson AM, Su WP, Mason T, O'Fallon WM, Gabriel SE (1997). "The epidemiology of morphea (localized scleroderma) in ... Frontal linear scleroderma (also known as en coup de sabre or morphea en coup de sabre) is a type of linear scleroderma ... ISBN 0-7216-2921-0. Peterson LS, Nelson AM, Su WP (1995). "Classification of morphea (localized scleroderma)". Mayo Clin. Proc ...
Fett, Nicole M. (2013-09-01). "Morphea (Localized Scleroderma)". JAMA Dermatology. 149 (9): 1124. doi:10.1001/jamadermatol. ... and systemic immunosuppressive drugs when the condition is part of systemic scleroderma[citation needed]. Localized treatment ... Sclerodactyly is a localized thickening and tightness of the skin of the fingers or toes that yields a characteristic claw-like ... Sclerodactyly is often preceded by months or even years by Raynaud's phenomenon when it is part of systemic scleroderma.[ ...
ISBN 0-7216-2921-0. Takehara K, Sato S (2005). "Localized scleroderma is an autoimmune disorder". Rheumatology. 44 (3): 274-9. ... "Neuromyelitis optica brain lesions localized at sites of high aquaporin 4 expression". Archives of Neurology. 63 (7): 964-968. ...
Valančienė G, Jasaitienė D, Valiukevičienė S (2010). "Pathogenesis and treatment modalities of localized scleroderma" (PDF). ... Scleroderma in pregnancy is a complex situation; it increases the risk to both mother and child. Overall, scleroderma is ... No single test for scleroderma works all of the time, hence diagnosis is often a matter of exclusion. Atypical scleroderma may ... The major scleroderma-related causes of death are: pulmonary hypertension, pulmonary fibrosis, and scleroderma renal crisis. ...
While exploring the role of ultraviolet A1 phototherapy in the treatment of localized scleroderma, she conducted a ... "Successful ultraviolet A1 phototherapy in the treatment of localized scleroderma: a retrospective and prospective study". The ...
... localized scleroderma, and eosinophilic fasciitis". Journal of the American Academy of Dermatology. 17 (4): 648-656. doi: ... The presentation is similar to that of scleroderma or systemic sclerosis. However, unlike scleroderma, eosinophilic fasciitis ... affects the deeper fascial layers, rather than the dermis; the characteristic and severe effects of scleroderma and systemic ...
The earliest symptoms may include a failure to thrive and a localized scleroderma-like skin condition. As a child ages past ... Scleroderma, a hardening and tightening of the skin on trunk and extremities of the body, is prevalent. People diagnosed with ... After prelamin A has been localized to the cell nuclear membrane, the C-terminal amino acids, including the farnesylated ...
"The First Presentation of Localized Scleroderma at Birth: Scleroderma as a Differential Diagnosis of Congenital Skin Lesion". ... The localized reaction takes 4-14 days to develop and can take months to heal. There has been rare reports of oral vitamin K ...
... and localized scleroderma. In addition, UV light, in particular UV‑B radiation, has been shown to induce cell cycle arrest in ...
An autoimmune mechanism is suspected, and the syndrome may be a variant of localized scleroderma, but the precise cause and ... specifically a variant of localized scleroderma. Several instances have been reported where more than one member of a family ... The syndrome often begins with a circumscribed patch of scleroderma in the frontal region of the scalp which is associated with ... Lakhani PK, David TJ (February 1984). "Progressive hemifacial atrophy with scleroderma and ipsilateral limb wasting (Parry- ...
... localized scleroderma, leukoplakia, vitiligo, and the cutaneous rash of Lyme disease can have a similar appearance. There is no ... In 1900 the concept was formed that scleroderma and LS are closely related, which continues to this day. In 1901, pediatric LS ... From 1913 to present, the concept that scleroderma is not closely related to LS also was formed. In 1920, Taussig established ...
Organizing pneumonia is usually preceded by some type of lung injury that causes a localized denudation or disruption in ... Ionizing radiations Inflammatory diseases Systemic lupus Rheumatoid arthritis (RA-associated COP) Scleroderma Bronchial ... and scleroderma. It most commonly presents in the 5th or 6th decade of life and it is exceedingly rare in children. ...
Diseases like scleroderma cause diffuse slowing of the bowel, leading to increased bacterial concentrations. More commonly, the ... Problems with motility may either be diffuse, or localized to particular areas. MMC impairment may be a result of post- ... Rose S, Young MA, Reynolds JC (September 1998). "Gastrointestinal manifestations of scleroderma". Gastroenterology Clinics of ...
... a chronic autoimmune connective tissue disease that can affect any part of the body Systemic scleroderma, also known as ... resistance response that occurs following an earlier localized exposure to a pathogen Systemic pesticide, a pesticide that ...
Linear scleroderma Localized discoid lupus erythematosus Localized morphea Lupus erythematosus panniculitis (lupus ... Localized acquired hypertrichosis Localized congenital hypertrichosis Longitudinal erythronychia Longitudinal melanonychia ... Acute cutaneous lupus erythematosus Atrophoderma of Pasini and Pierini (dyschromic and atrophic variation of scleroderma, ... Scleroderma-like reaction to taxanes Serum sickness-like reaction Steroid acne Steroid folliculitis Stevens-Johnson syndrome ...
Redness and scleroderma-like induration occurs on the skin. In addition, the mobility of the lips, hands, arms, and legs is ... Localized and disseminated cases are called papular mucinosis or lichen myxedematosus while generalized, confluent papular ... papular mucinosis Papular mucinosis of infancy Cutaneous mucinosis of infancy Nodular lichen myxedematosus Localized lichen ...
Restated, an inflammatory disorder primarily localized in the subcutaneous fat is termed a "panniculitis", a group of disorders ... connective tissue disorders such as lupus erythematosus or scleroderma; lymphoproliferative disease such as lymphoma or ...
... localizes to multivesicular bodies and the Golgi, and has a potential role in protein trafficking". Journal of Cell Science. ... "Impaired Smad7-Smurf-mediated negative regulation of TGF-beta signaling in scleroderma fibroblasts". The Journal of Clinical ...
As such clinical manifestations are localized to localization of contact. Transmission from animals to humans is supported; ... Sometimes these clinical manifestations are not ringworm but appear as impetigo, scleroderma or psoriasis. The lesions are ...
see below). Symptoms vary from localized warmth and erythema (redness) to joint pain and stiffness, to stinging pain that ... Inflammation of the bursae may also be caused by other inflammatory conditions such as rheumatoid arthritis, scleroderma, ...
The UK Scleroderma Study Group". British Journal of Rheumatology. 32 (5): 357-361. doi:10.1093/rheumatology/32.5.357. PMID ... The condition can cause localized pain, discoloration (paleness), and sensations of cold and/or numbness. When exposed to cold ... Secondary Raynaud's can occur due to a connective-tissue disorder such as scleroderma or lupus, injuries to the hands, ... Raynaud's phenomenon is the initial symptom that presents for 70% of patients with scleroderma, a skin and joint disease.[ ...
When the truncated prelamin A is localized to the nuclear envelope, it will not be processed and accumulates, leading to " ... Jansen, T; Romiti, R (2000). "Progeria infantum (Hutchinson-Gilford syndrome) associated with scleroderma-like lesions and acro ... Since WRNp's function depends on DNA, it is only functional when localized to the nucleus. Mutations that cause Werner syndrome ... with scleroderma-like lesions, loss of fat tissues, abnormal fat deposition leading to thin legs and arms, and severe ...
The protein encoded by this gene is a DNA binding and apoptosis-inducing protein and is localized in the nucleus. It is also a ... "C1D is a major autoantibody target in patients with the polymyositis-scleroderma overlap syndrome". Arthritis and Rheumatism. ...
New patches also appear over time and can be generalized over large portions of the body or localized to a particular area. ... Vitiligo is sometimes associated with autoimmune and inflammatory diseases such as Hashimoto's thyroiditis, scleroderma, ...
The initial changes are a break-down in the blood nerve barrier, followed by sub-perineurial edema and fibrosis; localized, ... hypothyroidism or scleroderma and psoriasis. There is increasing research that some forms of nerve entrapment, such as those in ...
An SNP (rs935332) within the human GPATCH2L region is related to scleroderma renal crisis (SRC), according to the validation ... GPATCH2L Homo sapiens protein is mainly localized to the nucleoplasm, according to GPATCH2L antibody staining from The Human ... of Anti-RNA Polymerase III Antibody-positive Systemic Sclerosis and Altered GPATCH2L and CTNND2 Expression in Scleroderma Renal ...
Similar results have also been witnessed in scleroderma, which involves hardening of the skin and inner organs. Skewing levels ... Additionally, skewed activation can also be localized to specific cell lineages. For example, one woman might have skewed ... and scleroderma. Autoimmune thyroid disease is a disease involving the thyroid gland. The immune system of those who have the ... "Skewed X chromosome inactivation in blood cells of women with scleroderma". Arthritis and Rheumatism. 52 (5): 1564-1570. doi: ...
Large-mouth diverticula are associated with scleroderma. Diverticular disease is more common in collagen disorders such as ... Clinical features of acute diverticulitis include constant abdominal pain, localized abdominal tenderness in the left lower ... Segmental colitis associated with diverticulosis (SCAD) is a condition characterized by localized inflammation of the colon ...
"MicroRNA expression abnormalities in limited cutaneous scleroderma and diffuse cutaneous scleroderma". Journal of Clinical ... "Evaluation of microRNA expression profiles that may predict recurrence of localized stage I non-small cell lung cancer after ...
2010). "The human core exosome interacts with differentially localized processive RNases: hDIS3 and hDIS3L". The EMBO Journal. ... J.E. Pope, JE (2002). "Scleroderma overlap syndromes". Current Opinion in Rheumatology. 14 (6): 704-10. doi:10.1097/00002281- ... Jablonska, S; Blaszczyk, M (1998). "Scleromyositis: a scleroderma/polymyositis overlap syndrome". Clinical Rheumatology. 17 (6 ... with one being localized in the cytoplasm (DIS3L1) and the other in the nucleus (DIS3). The second common associated protein is ...
IMMUNE RESPONSE IN LOCALIZED SCLERODERMA In localized scleroderma, your immune system attacks the skin and tissue underneath as ... CAUSES OF LOCALIZED SCLERODERMA When a healthy immune system reacts to a common virus like the cold or flu, it sends its ... What Causes Localized Scleroderma?. Our immune system protects us from getting sick. It clears out germs that may enter our ... Localized scleroderma is called an autoimmune disease.. Our immune system has many parts and players. The two main parts are ...
Localized fibrosing disorders include a spectrum of rare conditions that frequently begin in childhood. These lesions are ... Localized Fibrosing Disorders - Linear Scleroderma, Morphea, and Regional Fibrosis * Sections Localized Fibrosing Disorders - ... encoded search term (Localized Fibrosing Disorders - Linear Scleroderma%2C Morphea%2C and Regional Fibrosis) and Localized ... Localized scleroderma: response to occlusive treatment with tacrolimus ointment. Br J Dermatol. 2005 Jan. 152(1):180-2. [QxMD ...
The proposed study will investigate the esophageal involvement in the two forms of scleroderma (systemic and localized), ... Morphea, also known as localized scleroderma, is characterized by predominant skin involvement, with occasional involvement of ... localized scleroderma) patients. 56 and 31 newly and already diagnosed cases of SSc and morphea respectively were taken up for ... Localized scleroderma in children: clinical, diagnostic and therapeutic aspects. An Bras Dermatol. 2009;84:161-72. ...
There are neither sensitive nor specific laboratory tests for measuring disease activity in localized scleroderma (LS). ... Localized scleroderma (LS) is the most common type of scleroderma in children. LS differs from systemic sclerosis (SSc) by ... Uziel, Y., Feldman, B.M., Krafchik, B.R. et al. Increased serum levels of TGFβ1 in children with localized scleroderma. Pediatr ... Uziel Y, Krafchik BR, Silverman ED, Thorner PS, Laxer RM: Localized scleroderma in childhood: A report of 30 cases. Semin ...
Scleroderma is a rare condition that thickens your skin and tissue throughout your body. It can cause severe complications if ... Types of scleroderma. Healthcare providers classify scleroderma into two main types:. Localized scleroderma: Localized means ... Localized scleroderma symptoms. People with localized scleroderma usually only experience skin thickening. The thickened skin ... Its rare for localized scleroderma to affect your internal organs.. Systemic sclerosis symptoms. Systemic scleroderma can ...
Localized fibrosing disorders include a spectrum of rare conditions that frequently begin in childhood. These lesions are ... Localized Fibrosing Disorders - Linear Scleroderma, Morphea, and Regional Fibrosis * Sections Localized Fibrosing Disorders - ... encoded search term (Localized Fibrosing Disorders - Linear Scleroderma%2C Morphea%2C and Regional Fibrosis) and Localized ... Localized scleroderma: response to occlusive treatment with tacrolimus ointment. Br J Dermatol. 2005 Jan. 152(1):180-2. [QxMD ...
Juvenile localized scleroderma (jLS) is a rare condition that can impair health-related quality of life, especially as it ... Facilitating collaborations in pediatric localized scleroderma research: International validation of outcome measures. ... the international validation of the Localized Scleroderma Quality of Life Instrument (patient-reported; specific to jLS), ...
Morphea (Localized Scleroderma. Nicole M. Fett, MD. JAMA Dermatol. 2013;149(9):1124. doi:10.1001/jamadermatol.2013.5079. ... Peterson LS, Nelson AM, Su WP, Mason T, OFallon WM, Gabriel SE (1997). "The epidemiology of morphea (localized scleroderma) in ... Frontal linear scleroderma (also known as en coup de sabre or morphea en coup de sabre) is a type of linear scleroderma ... ISBN 0-7216-2921-0. Peterson LS, Nelson AM, Su WP (1995). "Classification of morphea (localized scleroderma)". Mayo Clin. Proc ...
Scleroderma is a disease that involves the buildup of fibrous tissue in the skin and elsewhere in the body. It also damages the ... Scleroderma is a disease that involves the buildup of fibrous tissue in the skin and elsewhere in the body. It also damages the ... Progressive systemic sclerosis; Systemic sclerosis; Limited scleroderma; CREST syndrome; Localized scleroderma; Morphea - ... Localized scleroderma (also called morphea) -- Often affects only the skin on the chest, abdomen, or limb but not usually on ...
B. Wörle; R. Hein; T. Krieg; M. Meurer Four patients with systemic scleroderma and 1 patient with localized scleroderma were ... Open the PDF for ,span class=search-highlight,Ciclosporin,/span, in Localized and Systemic Scleroderma - A Clinical Study in ... View article titled, ,span class=search-highlight,Ciclosporin,/span, in Localized and Systemic Scleroderma - A Clinical Study ...
Researchers determined the characteristics of inflammatory arthritis in children with localized scleroderma and their response ... Inflammatory Arthritis in Localized Scleroderma May Be Less Likely to Respond to Traditional Therapies. Rheumatology Advisor ... Characteristics of coexisting localized scleroderma and inflammatory arthritis [published online December 3, 2019]. Eur J ... Close more info about Inflammatory Arthritis in Localized Scleroderma May Be Less Likely to Respond to Traditional Therapies ...
Localized scleroderma [morphea]. L98.9. Disorder of the skin and subcutaneous tissue, unspecified. ...
Localized scleroderma [morphea] L94.1 Linear scleroderma L94.3 Sclerodactyly M32.0 Drug-induced systemic lupus erythematosus ...
There have been several reviews of scleroderma care over the last 5 years, but there are new developments in this field that ... of patients with scleroderma. These ulcers have the potential to resist conventional therapies. ... Scleroderma, Localized / complications * Scleroderma, Localized / diagnosis* * Skin Ulcer / drug therapy* * Skin Ulcer / ... Filling in the Gaps of Scleroderma Ulcer Care: A Review Adv Skin Wound Care. 2019 Dec;32(12):553-557. doi: 10.1097/01.ASW. ...
In scleroderma en coup de sabre (LScs) the atrophic lesion in frontoparietal area is the disease hallmark. Skin and ... Localized scleroderma is a rare disease, characterized by sclerotic lesions. A variety of presentations have been described, ... D. L. Tuffanelli, "Localized scleroderma," Seminars in Cutaneous Medicine and Surgery, vol. 17, no. 1, pp. 27-33, 1998. ... R. M. Laxer and F. Zulian, "Localized scleroderma," Current Opinion in Rheumatology, vol. 18, no. 6, pp. 606-613, 2006. ...
The term scleroderma is derived from the Greek words skleros (hard or indurated) and derma (skin) and it is used to describe a ... Localized cutaneous fibrosing disorders. Rheum Dis Clin North Am. 2013 May. 39(2):347-64. [QxMD MEDLINE Link]. ... Ferreli C, Gasparini G, Parodi A, Cozzani E, Rongioletti F, Atzori L. Cutaneous Manifestations of Scleroderma and Scleroderma- ... Clinical course of lung physiology in patients with scleroderma and interstitial lung disease: analysis of the Scleroderma Lung ...
The term scleroderma is derived from the Greek words skleros (hard or indurated) and derma (skin) and it is used to describe a ... Localized cutaneous fibrosing disorders. Rheum Dis Clin North Am. 2013 May. 39(2):347-64. [QxMD MEDLINE Link]. ... Ferreli C, Gasparini G, Parodi A, Cozzani E, Rongioletti F, Atzori L. Cutaneous Manifestations of Scleroderma and Scleroderma- ... Clinical course of lung physiology in patients with scleroderma and interstitial lung disease: analysis of the Scleroderma Lung ...
There are 2 main types of scleroderma: localized and systemic: * Localized scleroderma. There are 2 forms: * Morphea. This type ... Scleroderma in Children. What is scleroderma in children?. Scleroderma is an ongoing (chronic) disease that causes abnormal ... Localized scleroderma may affect patches of the skin on the torso, arms, legs, or head. ... What causes scleroderma in a child?. Scleroderma is thought to be an autoimmune disease. This means the symptoms are caused by ...
Dive into the research topics of Baseline Description of the Juvenile Localized Scleroderma Subgroup From the Childhood ... Baseline Description of the Juvenile Localized Scleroderma Subgroup From the Childhood Arthritis and Rheumatology Research ...
Localized Scleroderma (LoS) is an autoimmune connective tissue disease that affects skin and less commonly subcutaneous tissues ... Localized scleroderma clinical and ultrasound study Group.The localized scleroderma skin severity index and physician global ... LoS Localized scleroderma, LoSCAT Localized Scleroderma Cutaneous Assessment Tool, r Spearmans rank correlation coefficient ... Table 2 Health-related quality of life of patients with localized scleroderma. Full size table. ...
Learn about Scleroderma or find a doctor at Mount Sinai Health System. ... Localized Scleroderma. Localized scleroderma usually affects only the skin on the hands and face. Its progression is very slow ... General Outlook of Localized Scleroderma. Localized scleroderma nearly always carries a good prognosis and a normal lifespan. ... while localized scleroderma carries a good prognosis and normal lifespan. In children, localized scleroderma is three times ...
Scleroderma, Localized / complications * Scleroderma, Localized / epidemiology * Scleroderma, Localized / pathology * ...
Lupus and scleroderma are both autoimmune disorders, and they can occur at the same time. However, they are separate conditions ... Localized scleroderma affects the skin and underlying tissues. The patches may develop in one of two patterns. One pattern ... Scleroderma is primarily a skin disease, but when it affects other systems in the body, doctors call it systemic scleroderma, ... Systemic scleroderma. In scleroderma, the immune system attacks the skin, which causes. inflammation and the production of ...
Localized scleroderma. EBSCO DynaMed website. Available at: https://www.dynamed.com/condition/localized-scleroderma. Accessed ... What is scleroderma? Scleroderma Foundation website. Available at: http://www.scleroderma.org/site/PageNavigator/patients_ ... Scleroderma is a disease that affects the whole body. It can cause the skin, joints, and internal organs to thicken and stiffen ... Scleroderma. National Institute of Arthritis and Musculoskeletal and Skin Diseases website. Available at: https://www.niams.nih ...
Rheumatologists at UW Health offer the latest treatments for mild and severe forms of scleroderma. Find a doctor that can help ... Localized scleroderma rarely spreads and rarely affects your internal organs.. Localized scleroderma is either morphea or ... Scleroderma: Expert care for your skin and body. Consider UW Health, home of a leading scleroderma treatment and research team ... Diffuse scleroderma develops faster and causes more skin thickening. If you have diffuse scleroderma, you are at higher risk ...
Scleroderma, localized and systemic. *Systemic Sclerosis. *Myositis. *Sjögrens Syndrome. *Vasculitis. *Giant Cell Arteritis ...
Scleroderma is a rare autoimmune disorder made up of a group of diseases. There are 2 types of scleroderma: localized and ... Scleroderma Scleroderma is a rare autoimmune disorder. There are 2 types of scleroderma. One type affects the skin. The other ...
Morphea or localized scleroderma occurs in adults and children. Recent studies indicate that up to 20% of those with morphea, ... Related Rare Diseases: Warm Autoimmune Hemolytic Anemia, Systemic Scleroderma, Miller Fisher Syndrome, ... ...
Localized scleroderma. Morphea scleroderma: Oval patches of thick skin that are white in the middle and purple around the edges ... Localized scleroderma usually only affects the skin on the hands and face. Systemic scleroderma is more serious and affects ... Doctors often treat localized scleroderma with moisturizers or steroid creams. Oral medications, such as minocycline (Minocin ... Scleroderma. Scleroderma is a group of diseases that cause skin, and sometimes internal organs, to become hard and tight. In ...
Morphea or localized scleroderma.. *Pain. *Auto-inflammatory diseases.. DIAGNOSIS AND PERSONALIZED TREATMENT Comprehensive ...
  • Localized fibrosing disorders include several clinical and histopathological conditions that are similar to the skin involvement of systemic sclerosis , but the systemic features are absent. (medscape.com)
  • The term includes a variety of diseases, from localized scleroderma (LS) to systemic sclerosis. (hindawi.com)
  • Scleroderma is an aspect of systemic sclerosis , a systemic connective tissue disease that also involves subcutaneous tissue, muscles, and internal organs. (medscape.com)
  • In the prospective Autologous Stem Cell Transplantation International Scleroderma (ASTIS) trial, a phase 3 comparison of autologous HSCT with 12 successive monthly intravenous pulses of cyclophosphamide in 156 patients with early diffuse cutaneous systemic sclerosis, HCST was associated with higher treatment-related mortality than in the first year after treatment. (medscape.com)
  • Systemic scleroderma is also called systemic sclerosis . (mountsinai.org)
  • Scleroderma is primarily a skin disease, but when it affects other systems in the body, doctors call it systemic scleroderma, or systemic sclerosis. (medicalnewstoday.com)
  • Yes, a person can have lupus and scleroderma, or systemic sclerosis, at the same time. (medicalnewstoday.com)
  • Effective treatment of IPF, systemic sclerosis and localized scleroderma, represents an unmet medical need. (globenewswire.com)
  • 3 UVA1 is one of the most recent advances in phototherapy for localized scleroderma and systemic sclerosis, and has been used more extensively in Europe than North America or Asia. (skintherapyletter.com)
  • This disorder, which occurs most commonly in children and young adults, should not be confused with progressive systemic sclerosis or scleroderma (it literally means 'hard skin'), which is characterized by Raynaud phenomenon and systemic organ involvement (that's why we prefer the term morphea). (contemporarypediatrics.com)
  • Scleroderma, or progressive systemic sclerosis, is an autoimmune connective tissue disease that involves sclerotic changes of the skin and may involve internal organs. (logicalimages.com)
  • Systemic sclerosis sine scleroderma - Raynaud phenomenon and systemic involvement without skin sclerosis. (logicalimages.com)
  • The systemic sclerosis overlap syndrome is characterized by features of one of the scleroderma subsets with those of another autoimmune disease (eg, lupus erythematosus , dermatomyositis , Sjögren syndrome , and/or rheumatoid arthritis ). (logicalimages.com)
  • Scleroderma, also called systemic sclerosis, is an autoimmune disease that produces scars and patches of thick, rough, or scaly skin. (facty.com)
  • Frontal linear scleroderma (also known as en coup de sabre or morphea en coup de sabre) is a type of linear scleroderma characterized by a linear band of atrophy and a furrow in the skin that occurs in the frontal or frontoparietal scalp. (wikipedia.org)
  • Multiple lesions of en coup de sabre may coexist in a single patient, with one report suggesting that the lesions followed Blaschko's lines. (wikipedia.org)
  • In scleroderma en coup de sabre (LScs) the atrophic lesion in frontoparietal area is the disease hallmark. (hindawi.com)
  • Linear scleroderma en coup de sabre (LCsc) is a rare subset of LS. (hindawi.com)
  • Neurologic symptoms (e.g. seizures/epilepsy, headaches/migraines, stroke) and/or ophthalmology conditions such as uveitis may occur in LoS of the head or face (en coup de sabre subtype, progressive hemifacial atrophy) [ 3 ]. (biomedcentral.com)
  • Our patient has a rare subtype of linear morphea called 'en coup de sabre,' or 'stroke of the sword,' so-called because of the characteristic linear lesions that occur on the frontoparietal scalp and forehead. (contemporarypediatrics.com)
  • En coup de sabre has been associated with neurological complications, including facial palsy, facial hemiatrophy, and seizures. (contemporarypediatrics.com)
  • 2. Holland KE, Steffes B, Nocton JJ, et al: Linear scleroderma en coup de sabre with associated neurologic abnormalities. (contemporarypediatrics.com)
  • Scleroderma is more likely to cause serious complications if it affects your ability to breathe or process nutrition. (clevelandclinic.org)
  • What are possible complications of scleroderma in a child? (uhhospitals.org)
  • Complications of scleroderma vary depending on the type of the disease and how severe it is. (uhhospitals.org)
  • This form of scleroderma develops very slowly, causes few complications, and rarely spreads to other parts of the body. (healthstatus.com)
  • Other complications caused by systemic scleroderma are cancer, heart and kidney failure, pulmonary hypertension (high blood pressure in the lungs), and malabsorption (difficulty absorbing nutrients from food). (healthstatus.com)
  • Systemic scleroderma is a serious condition, while localized scleroderma carries a good prognosis and normal lifespan. (mountsinai.org)
  • Keep reading to learn about the similarities, differences, and relationship between lupus and scleroderma, including their symptoms, treatments, and prognosis for people with both conditions. (medicalnewstoday.com)
  • Morphea is a form of scleroderma that involves isolated patches of hardened skin on the face, hands, and feet, or anywhere else on the body, with no internal organ involvement. (wikipedia.org)
  • Bullous scleroderma, a rare form of scleroderma, may have altered angiogenic and lymphangiogenic characteristics. (cdlib.org)
  • This form of scleroderma causes linear patches of tough, waxy skin and cut-like scars that crease the forehead, neck, and scalp. (facty.com)
  • MUSC's Division of Rheumatology & Immunology is one of the top ranked programs in the country by US News and World Report and an international leader in the field of scleroderma clinical care and research. (globenewswire.com)
  • Dr Li, from Hackensack University Medical Center, discusses findings from her study on clinical features in pediatric localized scleroderma and how a rheumatologist can approach current challenges in. (consultant360.com)
  • To expand upon current measures in pediatric scleroderma, researchers identified clinical features that defined disease activity, as well as specificity and their relative importance in physician activity. (consultant360.com)
  • Ongoing research & development programs and expansion of clinical experiments in the field of scleroderma diagnostics & therapeutics are projected to create lucrative opportunities for market growth. (growthmarketreports.com)
  • Widespread scleroderma can occur with other autoimmune diseases, including systemic lupus erythematosus and polymyositis . (medlineplus.gov)
  • Some drugs, such as rituximab (Rituxan), mycophenolate mofetil (CellCept), and imatinib mesylate (Gleevac), used to treat certain autoimmune diseases and cancers may play a role in treating scleroderma. (mountsinai.org)
  • Our rheumatologists work with doctors across UW Health to treat the effects of severe scleroderma and other autoimmune diseases. (uwhealth.org)
  • Scleroderma may occur in tangent with other autoimmune diseases such as systemic lupus erythematosus and polymyositis. (healthstatus.com)
  • Autoimmune diseases can be difficult to diagnose due to the variety of symptoms, and scleroderma is no exception. (mountain-ice.com)
  • Systemic scleroderma, or sclerosis -- May affect large areas of skin and organs such as the heart, lungs, or kidneys. (medlineplus.gov)
  • Localized scleroderma (LoS) is an autoimmune disorder characterised by inflammation and sclerosis of the skin and less commonly subcutaneous fat, in some cases only affecting the fasciae, muscles and bones. (biomedcentral.com)
  • Systemic scleroderma can lead to the hardening of internal organs (sclerosis). (uwhealth.org)
  • If you have diffuse scleroderma, you are at higher risk for developing sclerosis. (uwhealth.org)
  • Hyperkeratosis, epidermal atrophy, vacuolization of basal cells, dermal edema and sclerosis, perivascular lymphohistiocytic infiltrate, multifocal vesiculation and dermo-epidermal fends are seen in the scleroderma skin. (cdlib.org)
  • Another, more serious variant of the disease, is called Systemic scleroderma, or sclerosis, and can affect large areas of the skin as well as other organs such as the heart, lungs, and kidneys. (healthstatus.com)
  • Phototherapy using longer-wavelength ultraviolet A (UVA) light (ie, UVA1, 340-400 nm) has proved beneficial for cutaneous lesions in scleroderma. (medscape.com)
  • The young man's lesion is consistent with linear morphea, or localized cutaneous scleroderma. (contemporarypediatrics.com)
  • Keloidal scleroderma is a very rare cutaneous presentation of scleroderma, in which keloidal plaques develop in normal or sclerotic skin. (logicalimages.com)
  • This may be due to their likelihood of having diffuse, rather than limited, cutaneous systemic scleroderma. (logicalimages.com)
  • An estimated 300,000 Americans have scleroderma, although the difficulty of diagnosis makes this number hard to pin down. (mountain-ice.com)
  • Atrophoderma of Pasini and Pierini (also known as "Dyschromic and atrophic variation of scleroderma," "Morphea plana atrophica," "Sclérodermie atrophique d'emblée") is a disease characterized by large lesions with a sharp peripheral border dropping into a depression with no outpouching, which, on biopsy, elastin is normal, while collagen may be thickened. (wikipedia.org)
  • Localized scleroderma is a rare disease, characterized by sclerotic lesions. (hindawi.com)
  • Lobomycosis can be classified into localized or multicentric forms, depending on the extent of skin lesions. (cdc.gov)
  • The proposed study will investigate the esophageal involvement in the two forms of scleroderma (systemic and localized), compare the same and address any need of upper gastrointestinal evaluation in morphea (localized scleroderma) patients. (biomedcentral.com)
  • The present study was designed to investigate the esophageal involvement in the systemic (SSc) and localized (morphea) forms of scleroderma and to compare the same. (biomedcentral.com)
  • Children are more likely than adults to develop localized forms of scleroderma. (medscape.com)
  • Localized scleroderma affects the skin and underlying tissues. (medicalnewstoday.com)
  • An autoimmune disease that affects the skin, muscles, blood vessels, and internal organs is known as scleroderma. (growthmarketreports.com)
  • A localized or systemic chronic and progressive autoimmune disorder characterized by thickening of the skin and the connective tissues. (embl.de)
  • In morphea scleroderma, patches of hard skin form and can last for years. (mountsinai.org)
  • If you have morphea scleroderma, you may have waxy patches of skin that vary in size and shape. (uwhealth.org)
  • In localized scleroderma, your immune system attacks the skin and tissue underneath as the 'foreign object. (scleroderma.org)
  • Scleroderma makes your body produce too much collagen, a protein that you need for healthy skin and tissue. (clevelandclinic.org)
  • Scleroderma can cause lots of symptoms and affect tissue throughout your body. (clevelandclinic.org)
  • Scleroderma makes patches of your skin and other tissue thicker than they should be. (clevelandclinic.org)
  • Scleroderma is a rare condition that makes your body produce tissue that's thicker than it should be. (clevelandclinic.org)
  • Scleroderma usually affects your skin, but can cause symptoms in any tissue throughout your body. (clevelandclinic.org)
  • Scleroderma is a disease that involves the buildup of fibrous tissue in the skin and elsewhere in the body. (medlineplus.gov)
  • An ECG may be done to find changes in the heart muscle tissue due to scleroderma. (uhhospitals.org)
  • Localized Scleroderma (LoS) is an autoimmune connective tissue disease that affects skin and less commonly subcutaneous tissues. (biomedcentral.com)
  • Scleroderma is an autoimmune disease - a condition caused when your immune system attacks your body's healthy tissue. (uwhealth.org)
  • Because scleroderma can affect your joints, bones, muscles and connective tissue, it is also considered a rheumatic condition. (uwhealth.org)
  • Systemic scleroderma affects your connective tissue and more parts of your body. (uwhealth.org)
  • Systemic scleroderma is more serious and affects connective tissue in many parts of your body, including internal organs. (adam.com)
  • Many early scleroderma symptoms are like those of other connective-tissue diseases, such as rheumatoid arthritis, lupus, and polymyositis. (adam.com)
  • Elizabeth Volkmann, MD, associate professor at UCLA, Director of the Scleroderma Program, and the codirector of UCLA's Connective Tissue Disease-Related Interstitial Lung Disease program, discusses treating. (consultant360.com)
  • Scleroderma is a connective tissue disease that is a type of autoimmune disorder causing changes in skin, muscle, blood vessels and internal organs. (healthstatus.com)
  • In the case of scleroderma, this rheumatic autoimmune disease tends to involve the thickening or tightening of the skin and the connective tissue under it, but many other symptoms may be present. (mountain-ice.com)
  • For example, tissue damage caused by excoriation can lead to localized infections and septicemia. (jcadonline.com)
  • However, a recent study has demonstrated that activated fibroblasts regulate tissue-localized transdifferentiation of regulatory T cells (Tregs) into T helper type 2 cell (Th2)-like cells through IL-33 in SSc lesional skin [ 2 ], suggesting that activated dermal fibroblasts amplify an aberrant immune response characteristic of SSc. (biomedcentral.com)
  • Morphea, also known as localized scleroderma, is characterized by predominant skin involvement, with occasional involvement of subjacent muscles and usually sparing the internal organs. (biomedcentral.com)
  • Internal involvement in localized scleroderma. (medscape.com)
  • Conversely, both fibronectin and collagen XVIII are increased in scleroderma skin, suggesting their involvement in the pathogenesis of bullous scleroderma. (cdlib.org)
  • Autoantibodies are frequently seen in localized scleroderma, particularly antinuclear antibodies and antibodies to single-stranded DNA and histones. (medscape.com)
  • The presence of anti-topoisomerase I, which is a specific type of antinuclear antibody, may indicate scleroderma. (medicalnewstoday.com)
  • CREST syndrome (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasias) refers to a subset of patients with limited scleroderma. (logicalimages.com)
  • Experts estimate that all types of scleroderma affect around 250 out of every 1 million people in the U.S. Around 100,000 people in the U.S. have systemic scleroderma. (clevelandclinic.org)
  • Some types of scleroderma affect only the skin, while others affect the whole body. (medlineplus.gov)
  • Juvenile localized scleroderma (jLS) is a rare condition that can impair health-related quality of life, especially as it affects other body parts besides the skin. (carragroup.org)
  • Disease characteristics among pediatric patients with coexisting localized scleroderma and inflammatory arthritis may vary widely, according to study results published in the European Journal of Rheumatology . (rheumatologyadvisor.com)
  • The most common scleroderma symptom is having patches or streaks of thickened, waxy skin. (clevelandclinic.org)
  • Linear scleroderma begins as a streak or line of hardened, waxy skin. (uwhealth.org)
  • Raised, waxy skin accumulations known as morphea are a hallmark of localized scleroderma. (facty.com)
  • Normally, extra collagen is cleaned up when the repair is done, but in localized scleroderma, your skin keeps making more and more collagen. (scleroderma.org)
  • If you have scleroderma, your immune system triggers your body's cells to produce too much collagen (a protein). (clevelandclinic.org)
  • Scleroderma is a rare disease of unknown etiology, characterized by thickening and hardening of skin resulting from increased collagen production. (hindawi.com)
  • Endostatin, an anti-angiogenic C-terminal fragment of collagen XVIII, has been recently reported to play a role in scleroderma pathogenesis, but collagen XVIII immunohistochemistry in scleroderma skin has still not been performed. (cdlib.org)
  • The apparently normal skin from the patient with scleroderma shows histological signs of the disease, with thick collagen fibers and lymphatic ectasia. (cdlib.org)
  • sufferers of scleroderma will experience a buildup of collagen in the skin and other organs and this is what leads to the symptoms of the disease. (healthstatus.com)
  • Systemic scleroderma is characterized as either limited or diffuse. (uwhealth.org)
  • As we've said, diagnosing scleroderma can be difficult because some of the symptoms are shared with other autoimmune and rheumatic disorders. (mountain-ice.com)
  • Joint pain is associated with scleroderma and many other rheumatic diseases. (facty.com)
  • Scleroderma is a chronic condition, which means you'll need to manage your symptoms for a long time (maybe the rest of your life). (clevelandclinic.org)
  • Severe fatigue is associated with scleroderma and many other chronic illnesses. (facty.com)
  • Approximately 2.5 million individuals are affected with scleroderma globally, which makes it a chronic disease. (growthmarketreports.com)
  • There have been reports of using Dupixent to treat adult alopecia areata , chronic urticaria , localized scleroderma , and even keloids ," she told Medscape. (medscape.com)
  • Localized scleroderma usually affects only the skin on the hands and face. (mountsinai.org)
  • This type of scleroderma usually affects both sides of the body, meaning if you have problems with your left arm, you'll have problems with your right arm, too. (adam.com)
  • Low-dose UVA phototherapy for treatment of localized scleroderma. (medscape.com)
  • Morphea en plaque is more common in adults. (medscape.com)
  • Rarely, if this type of scleroderma affects children or young adults, it may interfere with growth and cause severe deformities in the arms and legs. (mountsinai.org)
  • This summary discusses scleroderma in adults. (logicalimages.com)
  • As per the internal reports from Britain and Japan, the occurrence of scleroderma in adults is approximately 35 in 1 million . (growthmarketreports.com)
  • There are neither sensitive nor specific laboratory tests for measuring disease activity in localized scleroderma (LS). (biomedcentral.com)
  • 171 Linear scleroderma is a type of localised scleroderma which is an autoimmune disease characterized by a line of thickened skin which can affect the bones and muscles underneath it. (wikipedia.org)
  • Children and teenagers with active morphea (linear scleroderma, generalised morphea and mixed morphea: linear and circumscribed) may experience greater improvement of disease activity or damage with oral methotrexate plus prednisone than with placebo plus prednisone. (wikipedia.org)
  • The term scleroderma is derived from the Greek words skleros (hard or indurated) and derma (skin) and it is used to describe a disease characterized by progressive skin hardening and induration. (medscape.com)
  • Scleroderma is thought to be an autoimmune disease. (uhhospitals.org)
  • Scleroderma is an uncommon, complex, autoimmune disease. (mountsinai.org)
  • In children, localized scleroderma is three times more common than the systemic form of the disease. (mountsinai.org)
  • People with scleroderma may develop either a localized or a systemic (body-wide) form of the disease. (mountsinai.org)
  • Scleroderma is a disease that affects the whole body. (epnet.com)
  • Consider UW Health, home of a leading scleroderma treatment and research team, if you or a loved one are diagnosed with this autoimmune disease. (uwhealth.org)
  • Scleroderma is considered an autoimmune disease, meaning that the immune system mistakenly attacks the body's own tissues. (adam.com)
  • Histological and immunohistochemical aspects of skin biopsies are compared to normal skin from a patient without scleroderma and are correlated with the pathogenesis of the disease. (cdlib.org)
  • Variations of this disease may affect only the skin of the hands and face, this is referred to as localized scleroderma. (healthstatus.com)
  • Most reports point to a sclerodermalike disease rather than true systemic scleroderma. (logicalimages.com)
  • Due to this, research on the disease is limited and needs increased attention in order for doctors to better treat scleroderma. (mountain-ice.com)
  • About one-third of these patients have systemic scleroderma, the most serious form of the disease. (mountain-ice.com)
  • There are two types of the disease, localized and systemic scleroderma. (mountain-ice.com)
  • Living with scleroderma is difficult, as there is no drug that has been shown to stop or reverse the hardening and thickening of skin, the major symptom of the disease. (mountain-ice.com)
  • A term used to describe a variety of localized asymmetrical SKIN thickening that is similar to those of SYSTEMIC SCLERODERMA but without the disease features in the multiple internal organs and BLOOD VESSELS. (bvsalud.org)
  • Scleroderma is a long-term and rare disease that affects women more than men. (growthmarketreports.com)
  • No matter where you, your child, or a loved one are in your journey, or the type of scleroderma, the National Scleroderma Foundation can help you find your best path. (scleroderma.org)
  • Localized scleroderma (LS) is the most common type of scleroderma in children. (biomedcentral.com)
  • Which other symptoms you experience (and where they affect you) depends on which type of scleroderma you have. (clevelandclinic.org)
  • Scleroderma is a type of autoimmune disorder . (medlineplus.gov)
  • An antibody test may help show the type of scleroderma. (uhhospitals.org)
  • Symptoms differ from person to person and by type of scleroderma. (epnet.com)
  • Localized scleroderma is a milder type. (uwhealth.org)
  • Localized scleroderma is either morphea or linear in type. (uwhealth.org)
  • The other type… What is scleroderma? (familydoctor.org)
  • People with this type of scleroderma often have CREST syndrome. (adam.com)
  • Our aim is to report a rare case of bullous scleroderma, studying the presence of fibronectin and collagens type I, III and XVIII in sclerodermic skin. (cdlib.org)
  • Which type of systemic scleroderma is most likely to lead a patient to experience earlier organ damage? (consultant360.com)
  • Although the comorbid presentation of LS and inflammatory arthritis suggests an underlying systemic inflammatory component and perhaps even a shared immunopathology and a genetic component, additional studies are needed to clearly understand the relationship between localized scleroderma and inflammatory arthritis, and subsequently design the most appropriate treatment plan. (rheumatologyadvisor.com)
  • The growth of the market is attributed to the rising incidence of scleroderma cases and the increasing prevalence of genetic mutations along with the occurrence of extreme changes in the environment. (growthmarketreports.com)
  • Some people with scleroderma have a history of being around silica dust and polyvinyl chloride, but most do not. (medlineplus.gov)
  • Most people with scleroderma will develop only mild symptoms. (uwhealth.org)
  • Localized scleroderma: response to occlusive treatment with tacrolimus ointment. (medscape.com)
  • There is no specific treatment for scleroderma. (medlineplus.gov)
  • Many studies on scleroderma show some promise, but small sample sizes make it difficult to draw strong conclusions about treatment benefits. (mountsinai.org)
  • The drug was approved in Europe in 2007 for the treatment of skin ulcers related to scleroderma. (mountsinai.org)
  • Smaller units provide localized therapy, whereas whole-body treatment is best carried out using lie-down or standing UVA1 cabinets. (skintherapyletter.com)
  • The number of treatment sessions recommended for atopic dermatitis is usually 15 and for morphea or systemic scleroderma 20-40 treatments are given. (skintherapyletter.com)
  • 3. Weibel L, Sampai MC, Visentin MT, et al: Evaluation of methotrexate and corticosteroids for the treatment of localized scleroderma (morphoea) in children. (contemporarypediatrics.com)
  • Linear scleroderma is typically diagnosed in childhood and has the potential to affect limb development, so responsive treatment is important. (facty.com)
  • Currently, no drug is available and approved for the treatment of scleroderma. (growthmarketreports.com)
  • Healthcare providers usually refer to limited scleroderma with the acronym CREST syndrome. (clevelandclinic.org)
  • Limited scleroderma, sometimes referred to as CREST syndrome, affects the face, hands and feet. (uwhealth.org)
  • Localized scleroderma rarely spreads and rarely affects your internal organs. (uwhealth.org)
  • Scleroderma can cause small blood vessels in the kidneys to become narrowed. (medlineplus.gov)
  • Symptoms range from mild to life-threatening, and in severe cases, scleroderma can damage the blood vessels and organs. (facty.com)
  • Despite the name, limited scleroderma may narrow the blood vessels of the lungs and lead to lung scarring, which can cause shortness of breath. (facty.com)
  • Doctors believe scleroderma is caused by the immune system mistakenly attacking the body's own tissues. (adam.com)
  • Digital ulcers are a serious complication in 50% of patients with scleroderma. (nih.gov)
  • Several studies have demonstrated a reduction of new skin ulcers and accelerated healing of nondigital ulcers for certain scleroderma patients after taking Bosentan. (mountsinai.org)
  • M. Meurer Four patients with systemic scleroderma and 1 patient with localized scleroderma were treated with ciclosporin (CS) given in daily doses between 2.2 and 5.6 mg/kg body weight for 3-26 months. (karger.com)
  • Available at: http://www.scleroderma.org/site/PageNavigator/patients_whatis.html#.Wy58BVVKhxA. (epnet.com)
  • Most scleroderma patients, including children, have just one or two patches of hard or rough skin. (facty.com)
  • Although most patients have mild to moderate scleroderma, severe forms can injure the lungs, kidneys, and heart. (facty.com)
  • Scleroderma can lead to scarring of the skin, joints, and internal organs. (uhhospitals.org)
  • MCP, PIP and DIP joints destruction in patient with Scleroderma. (healthstatus.com)
  • Typically only affecting the skin, localized scleroderma can spread to muscles, bones, and joints, but not to the internal organs. (mountain-ice.com)
  • X-rays may show changes in bone, soft tissues, and organs caused by scleroderma. (uhhospitals.org)
  • Scleroderma is a group of diseases that cause skin, and sometimes internal organs, to become hard and tight. (adam.com)
  • Persons only affected with localized scleroderma have a better outlook than persons suffering from systemic scleroderma as the condition is localized to only a small area of the skin on the face and hands and rarely if ever spreads to internal organs. (healthstatus.com)
  • To diagnose scleroderma, your doctor will conduct a physical exam. (uwhealth.org)
  • It is not always easy to diagnose scleroderma. (adam.com)
  • Linear scleroderma and morphea can coexist in the same patient. (medscape.com)
  • Linear scleroderma tends to affect children and adolescents. (medscape.com)
  • Linear scleroderma. (medscape.com)
  • Linear scleroderma generally first appears in young children. (wikipedia.org)
  • patient data were identified from electronic medical records using diagnostic codes for morphea (ICD-9 701.0 and ICD-10 L94.0) and linear scleroderma (ICD-9 701.0 and ICD-10 L94.1). (rheumatologyadvisor.com)
  • There are two main forms of localized scleroderma: morphea and linear scleroderma. (mountsinai.org)
  • Linear scleroderma may also involve muscle or bone. (mountsinai.org)
  • linear scleroderma on the face or forehead. (mountain-ice.com)
  • Scleroderma symptoms can be intermittent or progressive depending on the form. (facty.com)
  • Characteristics of coexisting localized scleroderma and inflammatory arthritis [published online December 3, 2019]. (rheumatologyadvisor.com)
  • It is thought that the inflammation that accompanies the development of scleroderma initiates a keloidal response in those who are predisposed to keloid formation. (logicalimages.com)