Scleroderma, Localized
Scleroderma, Systemic
Scleroderma, Diffuse
Scleroderma, Limited
Raynaud Disease
Skin
Microscopic Angioscopy
Telangiectasis
Nails
Fibroblasts
Dermis
Autoantibodies
Antibodies, Antinuclear
Fibrosis
CREST Syndrome
Collagen Type I
Centromere Protein B
Facial Hemiatrophy
Lung Diseases, Interstitial
Connective Tissue Diseases
Bleomycin
Transforming Growth Factor beta
Skin Ulcer
Collagen
Connective Tissue Growth Factor
Pulmonary Fibrosis
Autoimmune Diseases
Hypertension, Pulmonary
Rheumatic Diseases
PUVA Therapy
Autoantigens
Silicones
Mixed Connective Tissue Disease
Smad3 Protein
Skin Diseases
Cells, Cultured
DNA Topoisomerases, Type I
Centromere
Skin Diseases, Vesiculobullous
Pulmonary Diffusing Capacity
Ulnar Artery
Penicillamine
Collagen Diseases
Gastrointestinal Diseases
Lupus Erythematosus, Systemic
South Australia
Breast Implants
Scalp Dermatoses
Smad7 Protein
Sclerosis
Respiratory Function Tests
Dermatomyositis
Ultraviolet Therapy
Pericardial Effusion
Immunosuppressive Agents
Severity of Illness Index
Carotid and femoral arterial wall mechanics in scleroderma. (1/62)
OBJECTIVE: Large-vessel arterial disease is increasingly recognized as a major cause of morbidity in autoimmune rheumatic disorders. Recent evidence suggests that scleroderma (systemic sclerosis, SSc) may be linked to altered fibrillin-1 metabolism associated with a defect in chromosome 15q. If this is the case, we may expect to see changes in the arterial wall mechanics of large vessels not clinically involved in the disease process. We undertook a study to determine whether the biomechanical properties and intima-media thickness (IMT) of the elastic carotid artery and the muscular femoral artery are altered in subjects with limited (lcSSc) and diffuse (dcSSc) cutaneous SSc. METHODS: Measurements of carotid and femoral wall mechanics were made in 33 patients with lcSSc, 19 patients with dcSSc and 21 control subjects, using a duplex scanner coupled to a Wall Track system. Their age, gender, body mass index, heart rate, systolic and diastolic blood pressures, presumed cardiovascular load, and plasma creatinine, fasting cholesterol, triglyceride and glucose concentrations were also measured. RESULTS: There was a progressive and significant reduction (P < 0.001) in the elastic properties of the carotid artery from the control group (compliance, 16.24 +/- 4.39 %mmHg(-1) x 10(-2)) to the lcSSc group (10.89 +/- 2.43 %mmHg(-1) x 10(-2)) to the dcSSc group (7.65 +/- 2.08 %mmHg(-1) x 10(-2)), even after adjustment for the systemic physiological and biochemical variables studied, which are known to influence the mechanics of arterial walls. There was no apparent difference between the groups in the mean elastic indices of the femoral artery and the IMT of the carotid and femoral arteries. CONCLUSION: The elastic properties of the carotid artery are significantly altered in SSc, and the two major subsets of SSc may be distinguished by their carotid artery biomechanics. This suggests that connective tissue abnormality occurs at sites not previously assessed. (+info)Seventeen-point dermal ultrasound scoring system--a reliable measure of skin thickness in patients with systemic sclerosis. (2/62)
OBJECTIVE: Our objective was to develop a 17-site ultrasound method of measuring skin thickness in patients with systemic sclerosis (SSc) and to assess its inter- and intra-observer variability. METHODS: Dermal thickness (using a 22 MHz ultrasound probe) was measured at 17 sites (corresponding to those assessed in the modified Rodnan skin score) in 39 patients with SSc (26 limited cutaneous, 13 diffuse) and 34 healthy controls. The sum of the thicknesses (at the 17 sites) and the maximal thickness were also documented. Because skin thickness varies between sites, each measurement was converted to a z-score. Inter- and intra-observer variability were assessed in 35 patients/33 controls, and 20 patients/15 controls respectively. RESULTS: Measurement precision was good for the dermal measurements-intraclass correlation coefficients at the 17 sites ranged from 0.65 to 0.94 for the inter-observer variability (0.86 for maximum thickness) and from 0.55 to 0.96 for the intra-observer variability (0.92 for maximum thickness). CONCLUSION: Our results suggest that the 17-point dermal ultrasound scoring system is extremely reliable and may therefore be a useful measure of outcome, including in clinical trials. (+info)Analysis of the 5' flanking region of the interleukin 10 gene in patients with systemic sclerosis. (3/62)
OBJECTIVES: Fibrosis, a feature of systemic sclerosis (SSc), is more severe in the diffuse compared with the limited disease variant. Interleukin 10 (IL-10) is an anti-inflammatory cytokine which reduces type 1 collagen mRNA levels in human fibroblasts. The 5' flanking region of the IL-10 gene is highly polymorphic, with three single base pair substitutions at position -1082(G/A), -819(C/T) and -592(C/A), which results in differential IL-10 production. The GCC/GCC genotype is associated with high IL-10 production while the ATA/ATA genotype with low production. We postulated that there would be a difference in IL-10 polymorphisms in patients with limited (lSSc) and diffuse (dSSc) disease. METHODS: Patients with limited (lSSc, n = 89) or diffuse (dSSc, n = 51) disease plus controls (n = 94) were recruited. DNA was isolated from peripheral blood and polymorphisms analysed using amplification refractory mutation system (ARMS) polymerase chain reaction (PCR). RESULTS: dSSc patients were less likely to carry the genotype indicative of high IL-10 production when compared with controls (controls vs dSSc; 29 vs 4%, chi2 = 15.7, 5 df, P = 0.005) and lSSc patients (lSSc vs dSSc; 21 vs 4%, chi2 = 17.5, 5 df, P = 0.002). There was no difference between control and lSSc patients. While there was no difference between controls and lSSc haplotypes, the GCC haplotype distribution did differ significantly between controls and dSSc patients (controls vs dSSc; 54 vs 36%, chi2 = 11.2, 2 df, P = 0.001). A significant difference was also observed between lSSc and dSSc haplotype distribution (lSSc vs dSSc; 48 vs 36%, chi2 = 13.5, 2 df, P < 0.001). CONCLUSION: We demonstrate that IL-10 genotypes associated with high IL-10 production are under-represented in dSSc. This may have implications in the disease pathology. (+info)Prevalence of systemic sclerosis in a French multi-ethnic county. (4/62)
OBJECTIVE: To assess the prevalence of systemic sclerosis (SSc) in a French multi-ethnic population and to examine ethnic differences. METHODS: This survey was conducted in Seine-Saint-Denis County, a suburb of Paris, home to 1,094,412 adults (>/=15 yr), among whom 26% are of non-European background with mainly northern and sub-Saharan African, Asian and Caribbean ancestries. The study period comprised the entire calendar year 2001. Patients were ascertained through four sources: public and private hospitals, general practitioners and community specialists, the French SSc patient support group, and the National Public Health Insurance System database. Only cases meeting either the 1980 ACR and/or LeRoy and Medsger's classification criteria were included and assigned to three clinical subsets: limited (normal skin) (l), limited cutaneous (lc) or diffuse cutaneous (dc) SSc. Capture-recapture (CR) analyses using log-linear modelling were performed to correct for incomplete case finding. RESULTS: We retained a total of 119 patients with SSc, including 15 extrapolated from inaccessible files. CR analysis estimated that 54.2 additional cases were missed by all the sources. The overall SSc prevalence (per million adults) was 158.3 (95% confidence interval, 129-187); those of lSSc, lcSSc and dcSSc were, respectively, 32.3 (16-48), 83.1 (66-101) and 42.9 (25-60); and respective values for Europeans and non-Europeans were 140.2 (112-170) and 210.8 (128-293). CONCLUSION: Regarding the heterogeneity of previously published estimates, this population-based survey using CR analysis might contribute to obtaining a better appraisal of SSc prevalence. Despite overlapping confidence intervals, the higher prevalence observed for non-Europeans could support potential influences of ethnic origin on the pathogenesis of SSc. (+info)Aortic stiffness in systemic sclerosis is increased independently of the extent of skin involvement. (5/62)
OBJECTIVE: To study the stiffness of large arteries in relation to the extent of skin and lung fibrosis, aortic distensibility was examined in patients with diffuse and limited systemic sclerosis (SSc). METHODS: Consecutive patients (55 with diffuse and 51 with limited SSc) without signs and symptoms of heart failure or a previous history of arterial hypertension underwent echocardiography and lung function tests. Aortic stiffness was determined non-invasively by aortic distensibility and aortic strain measurements in all patients and in 50 healthy subjects, matched for age and gender. RESULTS: Aortic distensibility in patients with either diffuse (2.03 +/- 0.26 x 10(-6) cm(2) dyn(-1)) or limited SSc (2.12 +/- 0.33) was similarly decreased compared with controls (2.49 +/- 0.36, P<0.001). Moreover, aortic strain was significantly reduced in both patient groups compared with controls, confirming that aortic stiffness is increased in SSc independently of the extent of skin involvement. Left ventricular performance was similar between patients and controls, while left ventricular mass and tricuspid systolic gradient were significantly increased in both SSc groups, the latter being associated with aortic stiffness in multivariate analysis. No association with serum levels of C-reactive protein or lung function abnormalities indicative of pulmonary fibrosis were found. CONCLUSIONS: Stiffness of the aorta is increased in patients with established SSc regardless of the extent of the inflammatory fibrotic process in the skin and lungs, suggesting that additional pathogenetic mechanisms contribute to the compromise of large arteries. (+info)Single-nucleotide polymorphisms in the SPARC gene are not associated with susceptibility to scleroderma. (6/62)
OBJECTIVE: SPARC (secreted protein, acidic and rich in cysteine) is a matricellular protein that modulates cell-cell and cell-extracellular matrix interactions. SPARC expression is restricted mainly to sites of tissue remodelling and wound repair, and is prominent in fibrotic disorders. Single-nucleotide polymorphisms (SNPs) in the SPARC gene are reportedly linked to scleroderma in four ethnic groups: Choctaw Indians, Caucasians, African Americans and Mexican Americans. We set out to reproduce and to positionally clone these disease associations in a set of UK Caucasian scleroderma patients and ethnically matched controls. METHODS: One hundred and twenty-one scleroderma subjects and 200 controls were genotyped by polymerase chain reaction with sequence-specific primers differing only in the 3' nucleotide corresponding to each allele of the biallelic SNPs. Scleroderma patients were analysed against controls and on the basis of their fibrosing alveolitis status as judged by high-resolution computed tomography evaluation and the extent of cutaneous involvement. RESULTS: Eight biallelic SNPs were genotyped: three from the last untranslated exon, which had been described previously, and an additional five novel SNPs: two in the promoter region, one in exon three and two in the 3' untranslated region. Six major haplotypes were constructed across all eight SNP positions. No significant differences in genotype, allele or haplotype frequency were observed between scleroderma and controls or within scleroderma subgroups. CONCLUSIONS: SNPs in the SPARC gene are not associated with susceptibility to scleroderma. This research adds to the genetic knowledge of the SPARC gene by identifying five novel SNPs spanning the whole gene and inserting these within the context of clearly defined haplotypes. (+info)HLA allelic variants encoding DR11 in diffuse and limited systemic sclerosis in Caucasian women. (7/62)
OBJECTIVE: We investigated HLA class II alleles in women with systemic sclerosis (SSc), a rare disease that preferentially affects women. METHODS: Specific alleles of DRB1, DQA1 and DQB1 were determined by DNA-based HLA typing for women with SSc (n = 102) and healthy women (n = 533). All study subjects were Caucasian. DRB1, DQA1 and DQB1 allele frequencies of women with SSc were compared with those of healthy women. RESULTS: Among women with SSc, 29.4% (30/102) and among healthy women 10.7% (57/533) had DRB1*11. Allele frequencies were compared for women with SSc and healthy women (each woman has two alleles). The allele frequency of DRB1*11 was 15.7% (32/204 alleles) in SSc women and 5.8% (62/1066 alleles) in healthy women (P = 0.000002). The increase of DRB1*11 was found both in diffuse (P = 0.0001) and limited SSc (P = 0.002) (allele frequencies 15.0 and 17.2%, respectively). Among women with diffuse SSc, there was a disproportionate increase of the DRB1*1104 allele (P = 0.0004) with no increase of DRB1*1101 (P = 1.00). In contrast, in limited SSc the strongest association was with DRB1*1101 (P = 0.008), with a less significant increase of DRB1*1104 (P = 0.04). CONCLUSIONS: An increase of DRB1*11 in SSc is consistent with other reports. Although present in both diffuse and limited SSc disease subsets, the increase was predominantly due to over-representation of DRB1*1104 in women with diffuse SSc. Women with limited SSc had a preponderance of DRB1*1101, the most common allele in healthy women. DRB1*1104 and DRB1*1101 differ by a single amino acid at position 86, where the former has valine and the latter glycine. (+info)Damage of cutaneous peripheral nervous system evolves differently according to the disease phase and subset of systemic sclerosis. (8/62)
OBJECTIVE: Evidence shows that peripheral nervous system (PNS) is involved in systemic sclerosis (SSc), but few morphological studies have assessed the ultrastructural pathological modifications. The aim was to study ultrastructural modifications of skin PNS fibres in SSc according to subsets [limited SSc (lSSc) and diffuse SSc (dSSc)] and phases (early and advanced) of the disease. METHODS: Skin biopsies were taken from the forearms of 23 SSc patients (11 lSSc and 12 dSSc) and 10 controls. Each biopsy was processed for transmission electron microscopy (TEM). RESULTS: At TEM, observation in skin from early lSSc, signs of inflammation were evident, while PNS fibres were not damaged. The microvascular wall showed hypertrophic endothelial cells bulging into the lumen. In advanced lSSc, fibrosis prevailed on inflammation and slight ultrastructural alterations of PNS fibres were evident in the papillary derma. In early dSSc, ultrastructural alterations of PNS fibres, similar to those observed in the advanced phase of lSSc, were found together with signs of inflammation and fibrosis. In advanced dSSc, in the papillary and reticular dermis PNS fibres were reduced and showed relevant ultrastructural alterations. CONCLUSIONS: In SSc, PNS ultrastructure damage is linked to the progression and severity of skin involvement. The alterations evolve from the early to the advanced phase mainly in the diffuse subset. In particular, the severe PNS lesions found in advanced lSSc are already present and widely diffuse in early dSSc and the microvascular involvement in early lSSc seems to precede the modification of the PNS in the skin. Thus, an early therapeutic approach can be useful to reduce the progression of PNS and skin damage in SSc patients. (+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.
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.
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.
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.
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.
"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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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!
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.
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.
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.
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.
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.
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.
Immunosuppressive agents are medications that decrease the activity of the immune system. They are often used to prevent the rejection of transplanted organs and to treat autoimmune diseases, where the immune system mistakenly attacks the body's own tissues. These drugs work by interfering with the immune system's normal responses, which helps to reduce inflammation and damage to tissues. However, because they suppress the immune system, people who take immunosuppressive agents are at increased risk for infections and other complications. Examples of immunosuppressive agents include corticosteroids, azathioprine, cyclophosphamide, mycophenolate mofetil, tacrolimus, and sirolimus.
In the field of medicine, "time factors" refer to the duration of symptoms or time elapsed since the onset of a medical condition, which can have significant implications for diagnosis and treatment. Understanding time factors is crucial in determining the progression of a disease, evaluating the effectiveness of treatments, and making critical decisions regarding patient care.
For example, in stroke management, "time is brain," meaning that rapid intervention within a specific time frame (usually within 4.5 hours) is essential to administering tissue plasminogen activator (tPA), a clot-busting drug that can minimize brain damage and improve patient outcomes. Similarly, in trauma care, the "golden hour" concept emphasizes the importance of providing definitive care within the first 60 minutes after injury to increase survival rates and reduce morbidity.
Time factors also play a role in monitoring the progression of chronic conditions like diabetes or heart disease, where regular follow-ups and assessments help determine appropriate treatment adjustments and prevent complications. In infectious diseases, time factors are crucial for initiating antibiotic therapy and identifying potential outbreaks to control their spread.
Overall, "time factors" encompass the significance of recognizing and acting promptly in various medical scenarios to optimize patient outcomes and provide effective care.
A Severity of Illness Index is a measurement tool used in healthcare to assess the severity of a patient's condition and the risk of mortality or other adverse outcomes. These indices typically take into account various physiological and clinical variables, such as vital signs, laboratory values, and co-morbidities, to generate a score that reflects the patient's overall illness severity.
Examples of Severity of Illness Indices include the Acute Physiology and Chronic Health Evaluation (APACHE) system, the Simplified Acute Physiology Score (SAPS), and the Mortality Probability Model (MPM). These indices are often used in critical care settings to guide clinical decision-making, inform prognosis, and compare outcomes across different patient populations.
It is important to note that while these indices can provide valuable information about a patient's condition, they should not be used as the sole basis for clinical decision-making. Rather, they should be considered in conjunction with other factors, such as the patient's overall clinical presentation, treatment preferences, and goals of care.
Hand dermatoses is a general term used to describe various inflammatory skin conditions that affect the hands. These conditions can cause symptoms such as redness, swelling, itching, blistering, scaling, and cracking of the skin on the hands. Common examples of hand dermatoses include:
1. Irritant contact dermatitis: A reaction that occurs when the skin comes into contact with irritants such as chemicals, soaps, or detergents.
2. Allergic contact dermatitis: A reaction that occurs when the skin comes into contact with allergens, such as nickel, rubber, or poison ivy.
3. Atopic dermatitis (eczema): A chronic skin condition characterized by dry, itchy, and inflamed skin.
4. Psoriasis: A chronic skin condition characterized by red, scaly patches that can occur anywhere on the body, including the hands.
5. Dyshidrotic eczema: A type of eczema that causes small blisters to form on the sides of the fingers, palms, and soles of the feet.
6. Lichen planus: An inflammatory skin condition that can cause purple or white patches to form on the hands and other parts of the body.
7. Scabies: A contagious skin condition caused by mites that burrow into the skin and lay eggs, causing intense itching and a rash.
Treatment for hand dermatoses depends on the specific diagnosis and may include topical creams or ointments, oral medications, phototherapy, or avoidance of triggers.
Systemic scleroderma
Scleroderma bermudense
Scleroderma
Antinuclear antibody
Mir-503 microRNA precursor family
Anti-centromere antibodies
Sclerodactyly
Progeria
Prostacyclin receptor
Eosinophilic fasciitis
Raynaud syndrome
Morphea
Uapaca bojeri
Relaxin
Sharon Runner
Colchicine
Anti-topoisomerase antibodies
Leri pleonosteosis
CREST syndrome
Anti-Scl-70 antibodies
Telangiectasia
List of MeSH codes (C17)
Crest
4-Aminobenzoic acid
Stiff skin syndrome
Dana Delany
Loss of heterozygosity
Caballeronia
Dieterich's disease
Breast-conserving surgery
Researchers interested in Scleroderma, Limited | Internal Medicine
Systemic scleroderma - Wikipedia
Clinical differences between idiopathic and scleroderma-related pulmonary hypertension
Scleroderma: MedlinePlus Medical Encyclopedia
Scleroderma Specialist. Symptoms & Treatment | Conditions | UW Health
Scleroderma: Symptoms, Causes & Treatment Options
Scleroderma Information | Mount Sinai - New York
Forms of Scleroderma - National Scleroderma Foundation
Scleroderma: Practice Essentials, Background, Pathophysiology
Limited Scleroderma Treatment Doctors in India | Ask Apollo
Treatment of Scleroderma Skin Ulcers Using Becaplermin Gel and Hydrocolloid Membrane in: Journal of the American Podiatric...
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Neurologic Involvement in Scleroderma en Coup de Sabre
What is Scleroderma? Scleroderma Causes & Symptoms
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Eustar - European Scleroderma Trials & Research Group
Scleroderma - SmartEngage
Scleroderma
Rogo EJ[au] - Search Results - PubMed
Category: Useful Links - Scleroderma Queensland
June is Scleroderma Awareness Month
Treatment Strategies for Systemic Scleroderma Identified
Limited systemic sclerosis (CREST syndrome): Video | Osmosis
Plus it
iBio Adds Biotherapeutic Product for Treatment of Fibrosis
Botox and Raynaud's - Scleroderma Queensland
CREST14
- There are two main types, limited disease (CREST syndrome) and diffuse disease. (medlineplus.gov)
- Limited scleroderma, sometimes referred to as CREST syndrome, affects the face, hands and feet. (uwhealth.org)
- Healthcare providers usually refer to limited scleroderma with the acronym CREST syndrome. (clevelandclinic.org)
- Limited scleroderma is sometimes called CREST syndrome. (scleroderma.org)
- CREST syndrome (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasias-although not all are needed for the disorder to be called CREST) is an older term used to describe this subset of limited cutaneous systemic sclerosis. (medscape.com)
- People with this type of scleroderma often have CREST syndrome. (adam.com)
- CREST syndrome (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasias) refers to a subset of patients with limited scleroderma. (logicalimages.com)
- Scleroderma or CREST syndrome is a chronic, auto immune disease which manifests as thick, dry, fibrous skin. (medindia.net)
- Scleroderma/CREST syndrome is classified as rheumatic and connective tissue disease. (medindia.net)
- CREST syndrome , also known as limited cutaneous systemic sclerosis , is an autoimmune condition, and its name is an acronym that stands for calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasias. (osmosis.org)
- CREST syndrome , also known as the limited cutaneous form of systemic sclerosis is a multisystem connective tissue disorder. (osmosis.org)
- 1 Systemic sclerosis is commonly divided into the limited form (formerly termed CREST) or the diffuse form. (skintherapyletter.com)
- Scleroderma / CREST syndrome (calcinosis cutis, Raynaud phenomena, esophageal dysfunction, sclerodactyly, and telangiectasia) is the most common overlap syndrome resulting in sclerodermatomyositis. (medpagetoday.com)
- I have CREST too, which is now known as Limited Scleroderma. (bcna.org.au)
Complications10
- Scleroderma is more likely to cause serious complications if it affects your ability to breathe or process nutrition. (clevelandclinic.org)
- This form of scleroderma can also cause complications like pulmonary arterial hypertension. (home-remedies-for-you.com)
- 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)
- Limited cutaneous systemic sclerosis - distal skin sclerosis, Raynaud phenomenon, frequent severe late-stage complications such as pulmonary hypertension and gastrointestinal involvement. (logicalimages.com)
- Scleroderma can attack any part of the body and damage internal organs, eventually leading to severe complications or even death. (dailyadvocate.com)
- Treatment for scleroderma is aimed at managing the symptoms and preventing complications. (welovelmc.com)
- While there is no cure for scleroderma, early diagnosis and treatment can help manage the symptoms and prevent complications. (welovelmc.com)
- and scleroderma renal crisis are the major complications. (msdmanuals.com)
- Renal and cardiovascular complications were the leading causes of death.Conclusion: Diffuse systemic sclerosis is a more serious disease than the limited form. (bvsalud.org)
Diffuse scleroderma9
- Lungs Some impairment in lung function is almost universally seen in patients with diffuse scleroderma on pulmonary function testing, but it does not necessarily cause symptoms, such as shortness of breath. (wikipedia.org)
- Digestive tract Diffuse scleroderma can affect any part of the gastrointestinal tract. (wikipedia.org)
- Diffuse scleroderma develops faster and causes more skin thickening. (uwhealth.org)
- If you have diffuse scleroderma, you are at higher risk for developing sclerosis. (uwhealth.org)
- In diffuse scleroderma , skin thickening occurs more rapidly and involves more skin areas than in limited disease. (scleroderma.org)
- In addition, people with diffuse scleroderma have a higher risk of developing "sclerosis" or fibrous hardening of the internal organs. (scleroderma.org)
- Although internal problems occur, they are less frequent and tend to be less severe than in diffuse scleroderma and are usually delayed in onset for several years. (scleroderma.org)
- However, persons with limited scleroderma, and occasionally those with diffuse scleroderma, can develop pulmonary hypertension, a condition in which the lung's blood vessels become narrow, leading to impaired blood flow through the lungs resulting in shortness of breath. (scleroderma.org)
- 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. (bvsalud.org)
Internal organs18
- Systemic scleroderma, or systemic sclerosis, is an autoimmune rheumatic disease characterised by excessive production and accumulation of collagen, called fibrosis, in the skin and internal organs and by injuries to small arteries. (wikipedia.org)
- Localized scleroderma rarely spreads and rarely affects your internal organs. (uwhealth.org)
- Systemic scleroderma can lead to the hardening of internal organs (sclerosis). (uwhealth.org)
- The internal organs are usually not affected, and persons with localized scleroderma rarely develop systemic scleroderma. (scleroderma.org)
- Scleroderma is an aspect of systemic sclerosis , a systemic connective tissue disease that also involves subcutaneous tissue, muscles, and internal organs. (medscape.com)
- There are two more versions - morpha and linear scleroderma - which affect the skin and may afflict the internal organs. (home-remedies-for-you.com)
- The word scleroderma literally means "hard skin," but the disease is much more than that, often affecting the internal organs with life-threatening consequences. (looktothestars.org)
- 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)
- 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)
- Scleroderma is a group of diseases that cause skin, and sometimes internal organs, to become hard and tight. (adam.com)
- Systemic scleroderma is more serious and affects connective tissue in many parts of your body, including internal organs. (adam.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)
- Scleroderma is a multi-system disease characterized by functional and structural abnormalities of small blood vessels, thickening of the skin and internal organs, and immune system activation. (cohencenters.com)
- An autoimmune disease that affects the skin, muscles, blood vessels, and internal organs is known as scleroderma. (growthmarketreports.com)
- Limited cutaneous scleroderma: This type of scleroderma affects only the skin on the face, neck, and limbs, and does not usually affect the internal organs. (welovelmc.com)
- Diffuse cutaneous scleroderma: This type of scleroderma affects the skin on the face, neck, and limbs, as well as the internal organs, and can progress more rapidly than limited cutaneous scleroderma. (welovelmc.com)
- Typically only affecting the skin, localized scleroderma can spread to muscles, bones, and joints, but not to the internal organs. (mountain-ice.com)
- The systemic form scleroderma involves the sclerosis (hardening) of internal organs. (drwilderman.com)
Pulmonary7
- Pulmonary arterial hypertension related to scleroderma (PAH-Scl) is associated with high morbidity and mortality as well as poorer response to therapy and worse outcomes compared with the idiopathic form of PAH (IPAH). (nih.gov)
- Scleroderma is an autoimmune disease that can affect left and right heart function directly through inflammation and fibrosis and indirectly through systemic and pulmonary hypertension. (nih.gov)
- Natural history of mild-moderate pulmonary hypertension and the risk factors for severe pulmonary hypertension in scleroderma. (jrheum.org)
- OBJECTIVE: To determine risk factors for developing pulmonary hypertension (PH) in patients with scleroderma (SSc, systemic sclerosis). (jrheum.org)
- Risk factors for progression of PH include older age, limited skin disease, and elevated pulmonary artery pressures at the time of initial evaluation. (jrheum.org)
- In limited SSc, a significant proportion of patients develop life-threatening pulmonary hypertension. (skintherapyletter.com)
- Mortality was similar between the two groups, and similar rates were also seen between the two for severe SSc-related end-organ damage, including pulmonary fibrosis, pulmonary hypertension , and scleroderma renal crisis. (medscape.com)
Rheumatic6
- Because scleroderma can affect your joints, bones, muscles and connective tissue, it is also considered a rheumatic condition. (uwhealth.org)
- Joint pain is associated with scleroderma and many other rheumatic diseases. (facty.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)
- 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)
- Scleroderma is one of our most challenging rheumatic diseases to treat. (medscape.com)
- Scleroderma, or progressive systemic sclerosis (PSS), an autoimmune rheumatic condition affecting the connective tissues , has a profound impact on oral health . (bvsalud.org)
Prognosis4
- Patients with limited systemic sclerosis have a better prognosis than those with the diffuse form. (wikipedia.org)
- Systemic scleroderma is a serious condition, while localized scleroderma carries a good prognosis and normal lifespan. (mountsinai.org)
- The aim is to coordinate and focus research activities, in order to improve treatment, quality of life and prognosis of patients with scleroderma. (eustar.org)
- Systemic sclerosis (SSc) differs from localized scleroderma in prognosis as well as clinical expression. (skintherapyletter.com)
Sclerosis28
- There are two major subgroups of systemic sclerosis based on the extent of skin involvement: limited and diffuse. (wikipedia.org)
- Systemic scleroderma, or sclerosis -- May affect large areas of skin and organs such as the heart, lungs, or kidneys. (medlineplus.gov)
- Systemic sclerosis has three subtypes - diffuse, limited and sine sclerosis. (clevelandclinic.org)
- Sine sclerosis causes limited sclerosis symptoms, but doesn't affect your skin. (clevelandclinic.org)
- Systemic scleroderma is also called systemic sclerosis . (mountsinai.org)
- localized scleroderma and systemic sclerosis (SSc). (scleroderma.org)
- Systemic scleroderma (systemic sclerosis) may affect the connective tissue in many parts of the body. (scleroderma.org)
- Systemic sclerosis is a complex and heterogeneous disease with clinical forms ranging from limited skin involvement (limited cutaneous systemic sclerosis) to forms with diffuse skin sclerosis and severe and often progressive internal organ involvement (diffuse cutaneous systemic sclerosis), and occasionally a fulminant course (fulminant systemic sclerosis). (medscape.com)
- Limited cutaneous systemic sclerosis involves areas distal to the elbows and knees but may involve the face and neck. (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)
- There is the limited systemic sclerosis or scleroderma which affects the hands, arms and face. (home-remedies-for-you.com)
- The next type is diffuse systemic sclerosis or scleroderma. (home-remedies-for-you.com)
- The term includes a variety of diseases, from localized scleroderma (LS) to systemic sclerosis. (hindawi.com)
- 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)
- The European Scleroderma Trials and Research group (EUSTAR) aims to foster the awareness, understanding and research on systemic sclerosis and its management throughout Europe and the rest of the world. (eustar.org)
- 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)
- Patients with systemic sclerosis, also known as systemic scleroderma, experience a sudden hardening, or fibrosis, of the skin. (medindia.net)
- A rare connective tissue autoimmune disorder, systemic sclerosis, also known as scleroderma, is difficult to treat. (medindia.net)
- Effective treatment of IPF, systemic sclerosis and localized scleroderma, represents an unmet medical need. (globenewswire.com)
- Early diagnosis and individualized therapy can be helpful, but treatment of systemic sclerosis is limited to symptom management. (globenewswire.com)
- Scleroderma, also called systemic sclerosis, is an autoimmune disease that produces scars and patches of thick, rough, or scaly skin. (facty.com)
- Dobrota et al 1 have analysed the EUSTAR (EULAR Scleroderma Trials and Research) systemic sclerosis (SSc, scleroderma) database using a subset of diffuse cutaneous SSc (dcSSc) to determine predictors of skin improvement over 1 year in randomised trials. (bmj.com)
- However, the ASTIS (Autologous hematopoietic stem cell transplantation vs. intravenous pulse cyclophosphamide in diffuse cutaneous systemic sclerosis) trial for stem cell transplantation versus cyclophosphamide had no upper limit of mRSS (inclusion criteria of mRSS of at least 15) and the mean baseline mRSS was 25-26. (bmj.com)
- Scleroderma, also known as systemic sclerosis, is a rare autoimmune disorder that causes the skin and connective tissues to harden and thicken. (welovelmc.com)
- Scleroderma, also known as systemic sclerosis, is a chronic connective tissue disease characterized by the hardening of skin and connective tissue. (drwilderman.com)
- In systemic sclerosis sine scleroderma, patients have systemic sclerosis-related antibodies and visceral manifestations of the disease but no skin tightening. (msdmanuals.com)
- Patients with both systemic sclerosis (SSc) and systemic lupus erythematosus (SLE) are more likely to be female, Black, and diagnosed with limited cutaneous SSc. (medscape.com)
Linear Scleroderma15
- Linear scleroderma begins as a streak or line of hardened, waxy skin. (uwhealth.org)
- There are two main forms of localized scleroderma: morphea and linear scleroderma. (mountsinai.org)
- Linear scleroderma causes bands of hard skin across the face or on a single arm or leg. (mountsinai.org)
- Linear scleroderma may also involve muscle or bone. (mountsinai.org)
- Linear scleroderma is a form of localized scleroderma which frequently starts as a streak or line of hardened, waxy skin on an arm or leg or on the forehead. (scleroderma.org)
- Linear scleroderma tends to involve deeper layers of the skin as well as the surface layers, and sometimes affects the motion of the joints, which lie underneath. (scleroderma.org)
- Linear scleroderma usually develops in childhood. (scleroderma.org)
- Linear scleroderma en coup de sabre (LCsc) is a rare subset of LS. (hindawi.com)
- Linear scleroderma is typically diagnosed in childhood and has the potential to affect limb development, so responsive treatment is important. (facty.com)
- In 1936, Dillehunt & Chuinard (6) described a case in which a lesion defined as "linear scleroderma" was associated with melorheostosis. (medicaljournals.se)
- 1) described the clinical and histological features that distinguish the cutaneous changes of LMS from those of linear scleroderma. (medicaljournals.se)
- The increased collagen bundles show a normal appearance in LMS, which is distinct from the abnormal appearance noted in linear scleroderma. (medicaljournals.se)
- Furthermore, the skin appendages are normal in LMS while they are atrophic in linear scleroderma. (medicaljournals.se)
- In addition, hypertrichosis does not usually occur in linear scleroderma. (medicaljournals.se)
- linear scleroderma on the face or forehead. (mountain-ice.com)
Systemic lupus erythem7
- Widespread scleroderma can occur with other autoimmune diseases, including systemic lupus erythematosus and polymyositis . (medlineplus.gov)
- Scleroderma may occur in tangent with other autoimmune diseases such as systemic lupus erythematosus and polymyositis. (healthstatus.com)
- This review aims to provide an assessment of the nature and extent of complement involvement in TMA associated with autoimmune diseases such as systemic lupus erythematosus, antiphospholipid syndrome, and scleroderma renal crisis. (jrheum.org)
- There were 24 people with rheumatoid arthritis, one with scleroderma, and one with systemic lupus erythematosus. (cdc.gov)
- The prevalence of scleroderma was 0.2% (RR 15.65, 95% CL 0.21-87.03) and the prevalence of systemic lupus erythematosus was 0.2% (RR 11.37, 95% CL 0.15-63.23). (cdc.gov)
- Conclusion: Although the association between scleroderma and silicosis has been more widely reported in the literature, the prevalence of rheumatoid arthritis was greater than the prevalence of scleroderma or systemic lupus erythematosus among a cohort of individuals with silicosis. (cdc.gov)
- Autoimmune disease overlap - Dermatomyositis can occur in conjunction with systemic lupus erythematosus (SLE), mixed-connective tissue disease, Sjögren syndrome, scleroderma, and rheumatoid arthritis. (medpagetoday.com)
Raynaud's5
- Systemic scleroderma and Raynaud's can cause painful ulcers on the fingers or toes, which are known as digital ulcers. (wikipedia.org)
- Local injection with botulinum toxin A (BTX-A) safely and effectively reduces Raynaud's phenomenon (RP) and nailfold small blood vessel, or capillary, abnormalities in women with scleroderma, according to data from a small study in China. (scleroderma.org.au)
- Raynaud's phenomenon is one of the most common symptoms of patients with scleroderma. (cohencenters.com)
- The report by Scleroderma & Raynaud's UK stated that in 2018 about 19,000 individuals were diagnosed with scleroderma in the UK. (growthmarketreports.com)
- This CIB is limited to a discussion of Secondary Raynaud's phenomenon resulting from the use of vibrating hand tools, referred to as vibration syndrome. (cdc.gov)
Affects10
- The limited form affects areas below, but not above, the elbows and knees with or without involvement of the face. (wikipedia.org)
- Localized scleroderma (also called morphea) -- Often affects only the skin on the chest, abdomen, or limb but not usually on the hands and face. (medlineplus.gov)
- Systemic scleroderma affects your connective tissue and more parts of your body. (uwhealth.org)
- Scleroderma usually affects your skin, but can cause symptoms in any tissue throughout your body. (clevelandclinic.org)
- Localized scleroderma only affects one part of your body (usually your skin). (clevelandclinic.org)
- Localized scleroderma usually affects only the skin on the hands and face. (mountsinai.org)
- 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 type of scleroderma affects large portions of the skin and can spread to other organs too. (home-remedies-for-you.com)
- 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)
- Scleroderma is a long-term and rare disease that affects women more than men. (growthmarketreports.com)
Raynaud1
- The results highlight the need for clinicians to recognize the SSc-SLE overlap syndrome and to watch for scleroderma organ involvement in patients with features of SLE, Raynaud syndrome, anti-U1-RNP antibody positivity, or an isolated nucleolar pattern of antinuclear antibodies. (medscape.com)
Rheumatoid4
- Many early scleroderma symptoms are like those of other connective-tissue diseases, such as rheumatoid arthritis, lupus, and polymyositis. (adam.com)
- The prevalence of rheumatoid arthritis was 5.2% (relative risk (RR) 2.73, 95% confidence limit (CL) 1.75-4.06). (cdc.gov)
- The findings of laboratory evaluation including complete blood count, liver function tests, urinalysis, rheumatoid factor, antinuclear antibody, anti-scl-70 antibody, anti-centromere antibody and anti-mitochondrial antibody were all within normal limits or negative. (medicaljournals.se)
- Silica exposure and/or silicosis has also been associated with autoimmune diseases such as lupus erythematosus, rheumatoid arthritis, scleroderma, and with glomerulonephritis. (cdc.gov)
Abnormalities3
- As scleroderma progresses, esophageal involvement from abnormalities in decreased motility may worsen due to progressive fibrosis (scarring). (wikipedia.org)
- Some laboratory abnormalities commonly seen in systemic scleroderma are frequently absent in the localized form. (scleroderma.org)
- Scleroderma is a relatively rare connective tissue disorder characterized by skin fibrosis, obliterative vasculopathy and distinct autoimmune abnormalities. (bvsalud.org)
Localized and systemic scleroderma2
- The survey study, " Barriers to care in juvenile localized and systemic scleroderma: an exploratory survey study of caregivers' perspectives ," was published in the journal Pediatric Rheumatology . (scleroderma.org.au)
- There are two types of the disease, localized and systemic scleroderma. (mountain-ice.com)
Clinical4
- 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)
- Scleroderma has a wide spectrum of clinical manifestations and severity, as well as a variable course. (cohencenters.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)
- Many other clinical conditions present substantial skin fibrosis and may be potentially confused with Scleroderma, sometimes leading to a wrong diagnosis (eosinophilic fasciitis, systemic amyloidosis, scleromyxedema, graft-versus-host disease, progeroid disorders, stiff skin syndrome). (bvsalud.org)
Cause of scleroderma3
- The cause of scleroderma is unknown. (medlineplus.gov)
- The exact cause of scleroderma is not known, but it is thought to be related to an overactive immune system. (welovelmc.com)
- Though the exact cause of scleroderma is yet to be determined, the disease itself involves the overproduction of collagen, a protein that binds skin and connective tissue. (drwilderman.com)
Cure for scleroderma2
- These drugs only help alleviate pain and discomfort caused by the symptoms, but there is no cure for scleroderma. (healthstatus.com)
- Although there is no cure for scleroderma, medications can reduce symptoms and help individuals lead active lives. (facty.com)
People with scleroderma3
- 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)
- People with scleroderma may develop either a localized or a systemic (body-wide) form of the disease. (mountsinai.org)
Autoimmune disorder2
- Scleroderma is a type of autoimmune disorder . (medlineplus.gov)
- Scleroderma is an autoimmune disorder . (clevelandclinic.org)
Collagen5
- Scleroderma makes your body produce too much collagen, a protein that you need for healthy skin and tissue. (clevelandclinic.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)
- 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)
- Namely, in scleroderma closely aggregated, coarse collagen bundles occupy the reticular dermis, together with hyalinized collagen bundles that replace the subcutaneous fat (9). (medicaljournals.se)
Interstitial lung di1
- I had a bilateral mastectomy in 2017 and was also told that radiation was off the table due to interstitial lung disease, from the scleroderma). (bcna.org.au)
Patients have systemic1
- About one-third of these patients have systemic scleroderma, the most serious form of the disease. (mountain-ice.com)
Severe6
- Our rheumatologists work with doctors across UW Health to treat the effects of severe scleroderma and other autoimmune diseases. (uwhealth.org)
- In some cases, the joints and muscles are affected, resulting in severe pain and limited mobility. (looktothestars.org)
- In more severe cases, the disease can affect organs throughout the body - this is known as systemic scleroderma - including the heart, kidneys, and blood vessels. (scleroderma.org.au)
- Symptoms range from mild to life-threatening, and in severe cases, scleroderma can damage the blood vessels and organs. (facty.com)
- Although most patients have mild to moderate scleroderma, severe forms can injure the lungs, kidneys, and heart. (facty.com)
- Severe fatigue is associated with scleroderma and many other chronic illnesses. (facty.com)
Toes2
- Vascular damage due to scleroderma can result in loss of fingers, toes and entire limbs. (looktothestars.org)
- White fingers and toes that become numb and painful in response to cold could be an early symptom of systemic scleroderma. (facty.com)
Morphea scleroderma2
- If you have morphea scleroderma, you may have waxy patches of skin that vary in size and shape. (uwhealth.org)
- In morphea scleroderma, patches of hard skin form and can last for years. (mountsinai.org)
Types of scleroderma affect2
- Some types of scleroderma affect only the skin, while others affect the whole body. (medlineplus.gov)
- 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)
Diagnosis and Treatment1
- Caregivers of children with juvenile scleroderma cited a lack of knowledge about the rare disease within the medical community as the primary barrier to a proper diagnosis and treatment, according to a new survey study. (scleroderma.org.au)
Blood vessels2
- Scleroderma can cause small blood vessels in the kidneys to become narrowed. (medlineplus.gov)
- 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)
Fibrous1
- Scleroderma is a disease that involves the buildup of fibrous tissue in the skin and elsewhere in the body. (medlineplus.gov)
Rheumatology1
- Methods: This was a prospective study of all the cases of scleroderma seen between January 2012 and June 2015 at the Rheumatology Clinic of the Olabisi Onabanjo University Teaching Hospital. (bvsalud.org)
Kidneys1
- For some patients, this hardening occurs only in limited areas, but for others, it quickly spreads across the body and to organs such as the heart, intestines and kidneys. (medindia.net)
Treatment for scleroderma1
- There is no specific treatment for scleroderma. (medlineplus.gov)
Occurs1
- Localized scleroderma occurs most commonly in children, while systemic scleroderma is more common in adults. (drwilderman.com)
Streaks1
- The most common scleroderma symptom is having patches or streaks of thickened, waxy skin. (clevelandclinic.org)
Rarely1
- Localized Scleroderma The changes, which occur in localized scleroderma, are usually found in only a few places on the skin or muscles, and rarely spread elsewhere. (scleroderma.org)
Skin ulcers2
- 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)
- The drug was approved in Europe in 2007 for the treatment of skin ulcers related to scleroderma. (mountsinai.org)
Subsets1
- Scleroderma is classified into several subsets defined by the degree of clinically involved skin. (cohencenters.com)
Waxy patches1
- Morphea is a form of localized scleroderma characterized by waxy patches on the skin of varying sizes, shapes and color. (scleroderma.org)
Typically nonspecific1
- Musculoskeletal The first joint symptoms that patients with scleroderma have are typically nonspecific joint pains, which can lead to arthritis, or cause discomfort in tendons or muscles. (wikipedia.org)
Symptom2
- 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)
- Though scleroderma manifests differently among patients, the most prominent symptom is hardening of the skin. (drwilderman.com)
Term scleroderma1
- 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)
International Scleroderma1
- EUSTAR is an international scleroderma research network acting under the umbrella of the World Scleroderma Foundation . (eustar.org)
Coup de sabre2
- Sometimes it forms a long crease on the head or neck, referred to as en coup de sabre because it resembles a saber or sword wound. (scleroderma.org)
- In scleroderma en coup de sabre (LScs) the atrophic lesion in frontoparietal area is the disease hallmark. (hindawi.com)