Sneddon Syndrome
Skin Diseases, Vascular
The natural course of Sneddon syndrome: clinical and magnetic resonance imaging findings in a prospective six year observation study. (1/10)
Sneddon syndrome (SS) is increasingly recognised as a cause of ischaemic stroke in young adults. As the natural course of SS is not well defined, the authors performed a prospective six year clinical and neuroradiological follow up study. Thirteen patients with definite diagnosis of SS (livedo racemosa, characteristic skin biopsy, and history of stroke) entered a follow up programme that consisted of clinical examinations, two magnetic resonance imaging (MRI) investigations, and a comprehensive laboratory follow up protocol. The most frequent clinical findings during follow up had been headache (62%) and vertigo (54%). Seven patients (54%) suffered from transient ischaemic attacks, however, completed stroke has not been obtained during follow up. Progression of white matter lesions detected in MRI were present in 10 of 13 patients. Laboratory follow up protocol revealed transient antiphospholipid antibodies in two subjects. This prospective six year follow up study suggests a low incidence of territorial stroke but outlines progressive leucencephalopathy in patients with SS. (+info)Protein Z deficiency in antiphospholipid-negative Sneddon's syndrome. (2/10)
BACKGROUND: Sneddon's syndrome is characterized by the association of ischemic cerebrovascular events and widespread livedo racemosa. The pathophysiology of Sneddon's syndrome remains elusive, but various prothrombotic abnormalities have been previously reported in this setting. Low levels of protein Z, a downregulator of coagulation, have been recently linked to an increased risk of arterial thrombosis. The purpose of this study was to investigate the levels of protein Z in a series of Sneddon's syndrome patients without circulating antiphospholipid antibodies in comparison with an age- and sex-matched control population. METHODS: Twenty-six patients and 78 healthy controls had determination of their protein Z blood levels by an enzyme-linked immunoassay test. Patients' thrombotic and vascular risk factors, including tobacco smoking, arterial hypertension, oral contraceptive agents, dyslipidemia, factor V Leiden, and factor II mutation were recorded. RESULTS: Protein Z plasma levels were significantly lower in patients (mean 1.47 mg/L) than in controls (mean 1.93 mg/L) (P=0.02). Prevalence of protein Z deficiency (level <1 mg/L) was significantly higher (P=0.001) among patients (31%) than among controls (3.8%). Factor V Leiden and heavy smoking were observed in 4 and 7 patients, respectively. CONCLUSIONS: Sneddon's syndrome could be viewed as the peculiar clinical expression of various and sometimes associated coagulation abnormalities. Low levels of protein Z may account, at least partly, for the thrombotic events observed in Sneddon's syndrome and shed a new light on its pathophysiology. Clinical implications for protein Z deficiency in this setting deserve further investigations. (+info)Sneddon's syndrome: clinical and laboratory analysis of 10 cases. (3/10)
Sneddon's syndrome is characterized by livedo reticularis and cerebrovascular lesions. We report the cases of women (mean age, 36.2 +/- 8.1 years) diagnosed with Sneddon's syndrome based on the presence of livedo reticularis and characteristic cerebrovascular findings. Seven of these patients had cerebral infarcts on cranial computed tomography scan. Antiphospholipid antibodies were positive in 6 of these cases. Three cases had abnormal levels of antithrombin III. Analyses of chromosome 6 revealed no abnormalities. In 3 of the cases, investigation of the pedigrees revealed autosomal dominant traits. Two cases had epilepsy, and 3 had migraine. One case with migraine also had myasthenia gravis. In addition, we detected inferior altudinal hemianopia in 2 cases, cognitive functional disorder in 3 and depression in 2. Based on these findings, the entire vascular, haematologic, neurologic, and dermatologic systems should be evaluated in patients diagnosed with Sneddon's syndrome. (+info)Clinical, neurovascular and neuropathological features in Sneddon's syndrome. (4/10)
Sneddon's syndrome (SS) is characterized by ischemic cerebrovascular episodes and livedo reticularis. It is more common in young women and can also be associated with valvulopathy, a history of spontaneous abortion, renal involvement and vascular dementia. We describe three cases of young women with this disease. The patients had repeated ischemic cerebral episodes, livedo reticularis and thrombocytopenia. CT and MRI showed strokes and cerebral atrophy. Autopsy in one of the patients revealed cerebral infarctions. Anticardiolipin antibodies were detected in two patients. Antiphospholipid antibodies may be found in some patients with ischemic cerebrovascular events and livedo reticularis. SS may thus be associated with antiphospholipid syndrome. We described three new cases of SS and discuss the pathophysiology of this disease. (+info)Sneddon's syndrome presenting with severe disabling bilateral headache. (5/10)
(+info)Seizures in primary antiphospholipid syndrome: the relevance of smoking to stroke. (6/10)
(+info)Sneddon syndrome associated with Protein S deficiency. (7/10)
(+info)Sneddon's syndrome: case report and review of its relationship with antiphospholipid syndrome. (8/10)
(+info)Sneddon syndrome is a rare medical condition characterized by the concurrence of livedo reticularis (a purplish, net-like discoloration of the skin) and recurrent strokes or transient ischemic attacks (TIAs). It primarily affects young to middle-aged women. The exact cause of Sneddon syndrome remains unknown, but it's thought to be an autoimmune disorder with potential involvement of the coagulation system.
The main diagnostic criteria for Sneddon syndrome are:
1. Livedo reticularis (fixed, persistent form)
2. One or more cerebrovascular events (strokes or TIAs)
Additional features may include cognitive impairment, migraine-like headaches, seizures, and other neurological symptoms. Diagnosis is often challenging due to its rarity and the need to exclude other conditions that can present with similar symptoms. Treatment typically involves anticoagulation therapy, antiplatelet agents, or immunosuppressive medications to manage symptoms and prevent further cerebrovascular events.
Vascular skin diseases are a group of medical conditions that affect the blood vessels in the skin. These disorders can be caused by problems with the structure or function of the blood vessels, which can lead to various symptoms such as redness, discoloration, pain, itching, and ulcerations. Some examples of vascular skin diseases include:
1. Rosacea: a chronic skin condition that causes redness, flushing, and visible blood vessels in the face.
2. Eczema: a group of inflammatory skin conditions that can cause redness, itching, and dryness. Some types of eczema, such as varicose eczema, are associated with problems with the veins.
3. Psoriasis: an autoimmune condition that causes red, scaly patches on the skin. Some people with psoriasis may also develop psoriatic arthritis, which can affect the blood vessels in the skin and joints.
4. Vasculitis: a group of conditions that cause inflammation of the blood vessels. This can lead to symptoms such as redness, pain, and ulcerations.
5. Livedo reticularis: a condition that causes a net-like pattern of discoloration on the skin, usually on the legs. It is caused by abnormalities in the small blood vessels.
6. Henoch-Schönlein purpura: a rare condition that causes inflammation of the small blood vessels, leading to purple spots on the skin and joint pain.
7. Raynaud's phenomenon: a condition that affects the blood vessels in the fingers and toes, causing them to become narrow and restrict blood flow in response to cold temperatures or stress.
Treatment for vascular skin diseases depends on the specific condition and its severity. It may include medications, lifestyle changes, and in some cases, surgery.
A syndrome, in medical terms, is a set of symptoms that collectively indicate or characterize a disease, disorder, or underlying pathological process. It's essentially a collection of signs and/or symptoms that frequently occur together and can suggest a particular cause or condition, even though the exact physiological mechanisms might not be fully understood.
For example, Down syndrome is characterized by specific physical features, cognitive delays, and other developmental issues resulting from an extra copy of chromosome 21. Similarly, metabolic syndromes like diabetes mellitus type 2 involve a group of risk factors such as obesity, high blood pressure, high blood sugar, and abnormal cholesterol or triglyceride levels that collectively increase the risk of heart disease, stroke, and diabetes.
It's important to note that a syndrome is not a specific diagnosis; rather, it's a pattern of symptoms that can help guide further diagnostic evaluation and management.
Sneddon's syndrome
IgA pemphigus
Sneddon
List of MeSH codes (C10)
Livedo reticularis
List of MeSH codes (C17)
Darrell Wilkinson
List of MeSH codes (C14)
List of syndromes
Face perception
List of skin conditions
List of diseases (S)
Myasthenia gravis
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Brookside (TV series)
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2002 in British television
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Sneddon's syndrome - Wikipedia
Case Report: Diagnosing Sneddon Syndrome - The Rheumatologist
Adenosine deaminase 2 deficiency: MedlinePlus Genetics
Volume 185 Issue 4 | Dermatology | Karger Publishers
Stroke: Symptoms, tests and treatment | Live Science
TIA - European Stroke Organisation
Idiopathic livedo reticularis Information | Mount Sinai - New York
Pediatric Antiphospholipid Antibody Syndrome: Practice Essentials, Background, Pathophysiology
Pustular Psoriasis: Background, Types of Pustular Psoriasis, Etiology
Internal Medicine
WTS database | WHO FCTC
DAR - Bologna Biocomputing Group
ADA2 Gene - Altmeyers Encyclopedia - Department Internal medicine
Livedo racemosa (Concept Id: C5441660) - MedGen - NCBI
Specific PHGKB|Rare Diseases PHGKB|PHGKB
Susac syndrome | MedLink Neurology
Detecting Multiple Sclerosis Mimics Early
Pesquisa | Portal Regional da BVS
Early-onset progressive neurodegeneration-blindness-ataxia-spasticity syndrome (Concept Id: C3809665) - MedGen - NCBI
Englisch - Deutsch
DeCS
Human Genome Epidemiology Literature Finder|Home|PHGKB
Rheumatology | Research & Publications | HCA Healthcare
Pediatric Antiphospholipid Antibody Syndrome: Background, Pathophysiology, Epidemiology
Korean Journal of Ophthalmology
PEPSIC - pepsic.bvsalud.org
Publikationen | Neurochirurgie Zürich
Conditions related to Headaches
Being Gwyneth Doherty-Sneddon | FMS Educational Research Development and Practice
Endinglines |.....Learn More, Be Sure!!
Doherty-Sneddon5
- Dr Gwyneth Doherty-Sneddon of the Department of Psychology will argue that during difficult cognitive activity (e.g. thinking of an answer to a challenging question) we often look away from the person we are in conversation with. (stir.ac.uk)
- Dr Doherty-Sneddon said: "We have recently shown that looking at someone's face can sometimes interfere with our abilities to remember and process information. (stir.ac.uk)
- Dr Doherty-Sneddon said: "Gaze aversion therefore promises to be a useful tool in many situations including pedagogical ones. (stir.ac.uk)
- Doherty-Sneddon G, Riby D, Calderwood L & Ainsworth L (2009) Stuck on you: Face-to-face arousal and gaze aversion in Williams syndrome. (stir.ac.uk)
- Doherty-Sneddon_Riby_et_al_32finalversion31.pdf] Publisher conditions require an 18 month embargo. (stir.ac.uk)
Racemosa3
- Sneddon's syndrome is a form of arteriopathy characterized by several symptoms, including: Severe, transient neurological symptoms or stroke Livedo reticularis, or livedo racemosa Sneddon's syndrome generally manifests with stroke or severe, transient neurological symptoms, and a skin rash (livedo reticularis). (wikipedia.org)
- Sneddon's syndrome may instead present with livedo racemosa, which involves larger, less organized patches of bluish-purple mottling of the skin. (wikipedia.org)
- The case was discussed with the patient's dermatologist, who agreed that based on evidence of a pattern consistent with livedo racemosa (see Figures 1-4, opposite) and characteristic biopsy findings, Sneddon syndrome was a concern. (the-rheumatologist.org)
Livedo reticularis3
- Livedo reticularis of the upper and lower extremities in a 15-year-old adolescent with primary antiphospholipid antibody syndrome. (medscape.com)
- Sneddon syndrome is a noninflammatory arteriopathy characterized by onset of livedo reticularis in the second decade and onset of cerebrovascular disease in early adulthood (summary by Bras et al. (nih.gov)
- Livedo reticularis with systemic involvement and stroke is SNEDDON SYNDROME. (bvsalud.org)
Subcorneal pustular2
- Sneddon-Wilkinson syndrome or subcorneal pustular dermatosis (SCPD): The disease follows a relapsing and remitting course that may develop into generalized pustular psoriasis. (medscape.com)
- Complete remission of skin lesions in a patient with subcorneal pustular dermatosis (Sneddon-Wilkinson disease) treated with anti-myeloma therapy: association with disappearance of M-protein. (myeloma.org.uk)
ADA21
- Deficiency of ADA2 (DADA2) is the first molecularly described monogenic vasculitis syndrome. (altmeyers.org)
Deficiency2
- Lee PY (2018) Vasculopathy, immunodeficiency, and bone marrow failure: the intriguing syndrome caused by deficiency of adenosine deaminase 2. (altmeyers.org)
- 1. AIDS: Acquired immune deficiency syndrome Acquired immunodeficiency syndrome 2. (cdc.gov)
Vasculopathy1
- The biopsy demonstrated histological findings characteristic of Sneddon syndrome, with obliterative vasculopathy, and changes that included fibrous replacement of medium caliber vascular structures with focal recanalization. (the-rheumatologist.org)
Cerebrovascular3
- Sneddon's syndrome is a progressive, noninflammatory arteriopathy leading to the characteristic skin condition and to cerebrovascular problems, including stroke, transient ischemic attack (TIA), severe but transient neurological symptoms thought to be caused by cerebral vasospasm, coronary disease and early-onset dementia. (wikipedia.org)
- In 1965, Dr. Sneddon first reported 6 patients with a distinct skin rash and cerebrovascular accidents (strokes).Sneddon's Syndrome was formerly understood to be a type of autoimmune disease called antiphospholipid syndrome, although it has been reclassified as a noninflammatory cerebrovascular disease. (wikipedia.org)
- Moyamoya syndrome is a progressive disorder that affects the blood vessels in the brain (cerebrovascular). (brainandnervecenter.com)
Secondary antiphospholipid antibody1
- The presence of antiphospholipid antibodies and a vaso-occlusive event superimposed on an underlying disease, such as SLE or malignancy, is a secondary antiphospholipid antibody syndrome. (medscape.com)
Susac6
- Susac syndrome presents with a triad of retinal arterial occlusion, deafness, and encephalopathy, although often not all of the components are evident at the onset. (medlink.com)
- Management of Susac syndrome is also discussed. (medlink.com)
- Susac syndrome is typically a triad of encephalopathy, retinopathy, and hearing loss, but may have an atypical presentation. (medlink.com)
- Susac syndrome is characterized by a triad of encephalopathy, retinopathy, and hearing loss. (medlink.com)
- The triad of clinical features in Susac syndrome is encephalopathy, retinopathy, and hearing loss. (medlink.com)
- the authors suggested classifying the disease course into suspected, incomplete, and complete Susac syndrome to facilitate early diagnosis. (medlink.com)
Abnormalities3
- Sneddon syndrome is a rare progressive disorder affecting the blood vessels characterized by the association of a skin condition and neurological abnormalities. (brainandnervecenter.com)
- Antiphospholipid syndrome is an acquired autoimmune disorder characterized by recurrent arterial or venous thrombosis and/or pregnancy losses, in the presence of persistently elevated levels of anticardiolipin antibodies and/or evidence of circulating lupus anticoagulant (these abnormalities are detected by blood tests). (endinglines.com)
- Goltz syndrome (focal dermal hypoplasia), a very rare syndrome that predominates in girls, is characterized by various ectodermal and mesodermal abnormalities. (tripod.com)
Gaze3
- My most recent work has investigated gaze aversion as cognitive load management in people with neurodevelopmental disorders, including autism and Williams syndrome. (ncl.ac.uk)
- In addition, they are beginning the first studies of gaze aversion as mental load management with special needs children, including children with William's syndrome and attention hyperactivity disorder. (stir.ac.uk)
- We investigate whether individuals with Williams syndrome (WS), associated with hyper-sociability and atypical face gaze, use GA to manage cognitive load and whether physiological arousal is associated with looking at faces. (stir.ac.uk)
Hypercoagulable2
- The patient's age, livedo and middle cerebral artery infarct, as well as her nonrevealing workup on repeat antiphospholipid antibody labs and hypercoagulable studies, were concerning for Sneddon syndrome. (the-rheumatologist.org)
- Hughes Syndrome/Antiphospholipid syndrome (APS) is a systemic autoimmune, hypercoagulable, thrombo inflammatory, and thrombosis and/or pregnancy complications syndrome caused by the persistent presence of antiphospholipid antibodies (APL) in plasma of patients with vascular thrombosis and/or pregnancy morbidity along with persistent anti-phospholipid antibodies (APLA), including lupus anticoagulant (LA), anti-β2-glycoprotein I (anti-β2GPI) and/or anti-cardiolipin (ACL) antibodies. (endinglines.com)
Antiphospholipid antibodies6
- About 40-60% of patients with the syndrome test positive for antiphospholipid antibodies. (wikipedia.org)
- Antiphospholipid antibodies associated with vaso-occlusive events without any underlying disease process is termed the primary antiphospholipid antibody syndrome (PAPS). (medscape.com)
- Preliminary classification criteria for "definite" antiphospholipid antibody syndrome were proposed in a report from the Eighth International Symposium on Antiphospholipid Antibodies and were published in Arthritis and Rheumatism . (medscape.com)
- Although features such as migraine headache, peripheral vasospasm, and thrombocytopenia were excluded from the published criteria, they were argued to be valid and useful clinical parameters in arriving at the diagnosis of antiphospholipid antibody syndrome in the clinical setting at the Ninth International Symposium on Antiphospholipid Antibodies. (medscape.com)
- In a consensus conference held at the 11th International Symposium on Antiphospholipid Antibodies, existing evidence on clinical and laboratory features of antiphospholipid antibody syndrome was appraised and amendments to the Sapporo criteria were proposed. (medscape.com)
- Antiphospholipid syndrome (APS) is caused by the body's immune system producing abnormal antibodies called antiphospholipid antibodies. (endinglines.com)
Metabolic Syndrome1
- Diabetes & Metabolic Syndrome: Clinical Research & Reviews , 102881. (otago.ac.nz)
Lesions1
- The advent of disease-modifying medications appears to have significantly altered the course of MS. The administration of disease-modifying medications in the clinically isolated syndrome has been repeatedly demonstrated to delay the progression to clinically definite MS. [ 2 , 3 ] Not only may this therapy decrease relapse rates and new MRI lesions, but it may also reduce the development of confirmed disability. (medscape.com)
Sneddon's3
- Both are generally found first in the extremities, both worsen in cold and either may occur without Sneddon's syndrome or any other systemic disease. (wikipedia.org)
- Sneddon's syndrome is a rare condition that is usually misdiagnosed. (wikipedia.org)
- Sneddon's syndrome most often becomes apparent in women in their thirties, though cases do occur in men and in children. (wikipedia.org)
Autoimmune disorder2
- Antiphospholipid antibody syndrome is a systemic autoimmune disorder that can manifest clinically as recurrent thrombosis. (medscape.com)
- Antiphospholipid syndrome is an autoimmune disorder of unknown cause. (endinglines.com)
Patient's1
- In general, the treatment of antiphospholipid antibody syndrome is individualized according to the patient's clinical status and history of thrombotic events. (medscape.com)
Manifestations1
- Sneddon syndrome: under diagnosed disease, complex clinical manifestations and challenging diagnosis. (hcahealthcare.com)
Catastrophic1
- Catastrophic antiphospholipid antibody syndrome is a multisystem failure secondary to thrombosis, infarction, or both and is characterized by microangiopathy on histopathologic examination. (medscape.com)
Williams2
- Feature salience and unfamiliar face processing in Williams syndrome and autism. (bvsalud.org)
- Results: In study 1, whilst WS was associated with general hypo-arousal, face arousal effects were found for both Williams syndrome and typically developing participants. (stir.ac.uk)
Disorder1
- Tolosa-Hunt syndrome is a rare disorder characterized by severe periorbital headaches, along with decreased and painful eye movements (ophthalmoplegia). (brainandnervecenter.com)
Diagnosis2
- [ 1 ] The criteria for the diagnosis of pediatric antiphospholipid antibody syndrome have not yet been validated. (medscape.com)
- In this article, the author describes the clinical features, pathology, and diagnosis of this syndrome as well as atypical presentations. (medlink.com)
Clinically2
- In partial or variant forms of this syndrome, only one or two of the three components are clinically manifest. (medlink.com)
- Therefore, in order to address the needs of clinicians and to expand the data for future research, the discussion included definitions on features of antiphospholipid antibody syndrome that were not included in the updated criteria for use clinically and in research. (medscape.com)
Etiology1
- Spontaneous Osteonecrosis of the Jaw During Bisphosphonate Therapy: An Unusual Etiology of the Numb Chin Syndrome. (myeloma.org.uk)
Thrombosis3
- In patients with primary antiphospholipid antibody syndrome with venous thrombosis, the initial treatment consists of heparin followed by warfarin or low molecular weight heparin. (medscape.com)
- In patients with primary antiphospholipid antibody syndrome with arterial thrombosis or infarction, many physicians administer antiplatelet therapy in the absence of other risk factors, but the use of anticoagulants is controversial. (medscape.com)
- These were published as the "International Consensus Statement on an Update of the Classification Criteria for Definite Antiphospholipid Antibody Syndrome (APS)" in J Thrombosis Haemost . (medscape.com)
Defects1
- CHILD syndrome (congenital hemidysplasia with ichthyosiform erythroderma and limb defects) is a very rare syndrome. (tripod.com)
Clinical features1
- If the clinical features suggest an antiphospholipid antibody syndrome, a thorough evaluation to detect the presence of at least one of these antibodies is essential. (medscape.com)
Conditions1
- AIDS-like syndrome: AIDS-like disease (illness) (syndrome) ARC AIDS-related complex Pre-AIDS AIDS-related conditions Prodromal-AIDS 3. (cdc.gov)
Facilitate1
- The purpose of the report was to define the essential features of antiphospholipid antibody syndrome in order to facilitate studies of treatment and causation. (medscape.com)
Type1
- Oral facial-digital syndrome type 1 ( OFD-1 ) is inherited in an X-linked dominant pattern. (tripod.com)
Apparent1
- The problem that dogs the work of all of those treating patients with antiphospholipid syndrome (APS) is the apparent lack of knowledge of the syndrome, both by the general public, as well as by swaths of the medical fraternity. (the-rheumatologist.org)
Patients1
- Vasospastic or vaso-occlusive events can occur in any organ system in patients with antiphospholipid antibody syndrome. (medscape.com)
Primary1
- Antiphospholipid syndrome can be primary or secondary. (endinglines.com)
Involves1
- Asymptomatic Chiari I syndrome should be ignored (this involves most Chiari malformations). (brainandnervecenter.com)