Conclusion Inspite of most previous reports, which indicate ischemic stroke as an usual central nervous system manifestation, SS could be presented with hemorrhagic stroke even in the initial phase of presentation. [rcm.mums.ac.ir]

Intracerebral hemorrhage (ICH) is unusual in Sneddon's syndrome and has not been reported as the presenting complaint. [ncbi.nlm.nih.gov]

• Rigor

  Sneddon's syndrome patients have a worse prognosis and may represent a subgroup of patients who demands more rigorous follow-up. [ncbi.nlm.nih.gov]

  Sneddon’s syndrome patients have a worse prognosis and may represent a subgroup of patients who demands more rigorous follow-up. [journal.einstein.br]

• Pneumonia

  Subcorneal pustular dermatosis associated with Mycoplasma pneumoniae infection. J Am Acad Dermatol. 2014 Sep. 71 (3):e85-6. [Medline]. Papini M, Cicoletti M, Landucci P. Subcorneal pustular dermatosis and mycoplasma pneumoniae respiratory infection. [emedicine.medscape.com]

  She died on November 23.Ą¤Clinical diagnosis: Parkinsonism (PSP s/o), hypoxic encephalopathy, pneumonia and gastric ulcer.Ą¤Pathological findings: aspiration pneumonia with organization, gastric ulcer (scar) + gastrointestinal tract bleeding, atrophy [knp.gr.jp]

  Dermatomyositis Rheumatoid arthritis Polyarteritis nodosa Granulomatosis with polyangiitis Temporal arteritis Sjogren syndrome Neurological disease Reflex sympathetic dystrophy Multiple sclerosis Parkinson disease Infection Tuberculosis Syphilis Mycoplasma pneumonia [dermnetnz.org]

  CSF IgG abnormality-positive MS patients had a significantly higher frequency of anti-C. pneumoniae IgG antibodies compared with CSF IgG abnormality-negative MS patients, although there was no difference in the frequency of anti-C. pneumoniae IgG antibodies [yoran.office.ehime-u.ac.jp]

• Hypertension

  Hypertension is the only risk factor significantly associated with a more severe course of the disease; no medication proved effective. [ncbi.nlm.nih.gov]

  Clinical manifestations may include hypertension and associated coagulopathies. The underlying pathophysiology of this syndrome remains obscure, yet increasing recognition of this entity may further ongoing investigations. [medlink.com]

  Diagnosis confirmation The diagnosis depends on the constellation of livedo, TIA or CVA, and hypertension. [dermatologyadvisor.com]

• Vascular Disease

  Sneddon's syndrome is a rare vascular disease affecting mainly skin and brain arterioles leading to their occlusion due to excessive endothelial proliferation. [ncbi.nlm.nih.gov]

  Capillary vessel disease Skin disease Complex syndrome Nervous system diseases Vascular disease Keyword (es) Hombre Livedo reticularis Medicina Sneddon síndrome Aparato circulatorio patología Capilar sanguíneo patología Piel patología Síndrome complejo [pascal-francis.inist.fr]

  Mechanisms of kidney disease in Sneddon’s syndrome: case report and literature review. [3] F. Macário, M.C. Macário, A. Ferro, F. Gonçalves, M. Campos, A. Marques. Sneddon’s syndrome: a vascular systemic disease with kidney involvement?. [revistanefrologia.com]

  Abstract Vasospastic diseases are peripheral vascular disorders caused by vasospasm, which means a reversible localized or diffuse vasoconstriction of arteries or smaller blood vessels. [link.springer.com]

  The Journal of Headache and Pain 2009 10 :109 https://doi.org/10.1007/s10194-009-0109-3 © Springer-Verlag 2009 Received: 7 December 2008 Accepted: 4 February 2009 Published: 14 March 2009 Abstract Sneddon’s syndrome is a rare vascular disease affecting [thejournalofheadacheandpain.springeropen.com]

• Cyanosis

  What you should be alert for in the history Aside from livedo reticularis or racemosa, and possibly digital infarcts/limb ischemia, Raynaud phenomenon, or acral cyanosis in some patients, there are no dermatologic findings in the history or physical [dermatologyadvisor.com]

  Trauma or alterations in coagulation should be ruled out in the initial evaluation, as well as cyanosis secondary to hypoxemia or methemoglobinemia. [elsevier.es]

  Ruddiness of the complexion and occasionally cyanosis, affecting mostly the face, neck, and distal parts of the extremities, may occur. Less frequent changes are telangiectasias and bleeding gums. Rosacea and purpura can be present. [emedicine.medscape.com]

• Petechiae

  Idiopathic thrombocytopenic purpura mostly occurs with an onset of petechiae and/or ecchymoses on the skin and mucous membranes. [emedicine.medscape.com]

  4, 2010) Dermoscopy of eccrine acrospiroma masquerading as nodular malignant melanoma (številka: 4, 2010) Managing scleromyxedema with intravenous immunoglobulin: acute worsening of scleromyxedema with biclonal gammopathy (številka: 4, 2010) Facial petechiae [dlib.si]

• Arthralgia

  The patient denied any episodes of thrombosis, photosensibility, arthralgias, malar rash or oral ulcers. [scielo.br]

  He reported arthralgia in the proximal interphalangeal joints of both hands and a history of photosensitivity (although not recent). [elsevier.pt]

  Mixed cryoglobulinemias present with the classic Melzer triad of purpura, arthralgias, and weakness in 25-30% of patients. [emedicine.medscape.com]

• Muscle Cramp

  cramps acral enlargement Johnson Munson syndrome Chromosome Xq28 deletion syndrome Hunter-McAlpine syndrome Neuroaxonal dystrophy, infantile Branchiootic syndrome Hereditary sensory and autonomic neuropathy type 2 Ring chromosome 12 Ichthyosis, leukocyte [checkrare.com]

• Muscle Spasm

  spasms Hirschsprung disease polydactyly heart disease Amino aciduria with mental deficiency, dwarfism, muscular dystrophy, osteoporosis and acidosis Refsum disease Spastic paraplegia 25 Camptodactyly-ichthyosis syndrome BRCA1 hereditary breast and ovarian [checkrare.com]

• Renal Insufficiency

  We investigated 2 patients with Sneddon's syndrome, elevated anticardiolipin antibodies and systemic complications, which included stroke, habitual abortions, cardiac valvular lesions, acrocyanosis, hypertension and renal insufficiency. [ncbi.nlm.nih.gov]

  Keywords Livedo reticularis ischemic stroke intimal proliferation renal insufficiency malignant hypertension To read this article in full you will need to make a payment References Zelger B Sepp N Stockhammer G Dosch E Hilty E Ofner D Aichner F Fritsch [ajkd.org]

  Clinical features associated with SS range from neurological symptoms, such as headache or cognitive impairment, to heart valve anomalies and renal insufficiency [2–5]. [journals.plos.org]

  Antiphospholipid antibodies are associated with an increased risk for chronic renal insufficiency in patients with lupus nephritis. Am J Kidney Dis. 2004 Jan. 43(1):28-36. [Medline]. Nochy D, Daugas E, Huong DL, et al. [emedicine.medscape.com]

• Kidney Failure

  Goodpasture syndrome (GPS), also known as anti-glomerular basement membrane disease, is a rare autoimmune disease in which antibodies attack the basement membrane in lungs and kidneys, leading to bleeding from the lungs and kidney failure. [en.wikipedia.org]

  Complications include: Kidney failure. This can result from decreased blood flow to your kidneys. Stroke. [mayoclinic.org]

• Stroke

  Conclusion Inspite of most previous reports, which indicate ischemic stroke as an usual central nervous system manifestation, SS could be presented with hemorrhagic stroke even in the initial phase of presentation. [rcm.mums.ac.ir]

  CONCLUSION: AECA were frequently found in patients with Sneddon's syndrome, in contrast to the patients with stroke without livedo. [ncbi.nlm.nih.gov]

  The association between migraine and juvenile stroke: a case–control study. Headache 2003 ; 43: 90 – 5. Google Scholar | Crossref | Medline | ISI 9. [doi.org]

• Headache

  The proportion with headache in this second group is 58% (102 individuals), with headache described as migraine in 28 (27.5%) of the headache subjects, including six with migraine with aura. [ncbi.nlm.nih.gov]

  Headache: Headache. 2002;42:352-355. Bottin L, Frances C, De Zuttere D, et al. Sneddon Syndrome without Antiphospholipid Antibodies in 55 Cases: Clinical, Radiological and Pathophysiological Features (S43. 005). [rcm.mums.ac.ir]

  J Neurol Neurosurg Psychiatry 74:542–544 PubMed Central PubMed View Article Google Scholar Headache Classification Subcommittee of The International Headache Society (2004) The International Classification of Headache Disorders, 2nd edn. [thejournalofheadacheandpain.springeropen.com]

  Headache Classification Committee of the International Headache Society. Classification and diagnostic criteria for headache disorders cranial neuralgias and facial pain. Cephalalgia 1988 ; 8 ( Suppl. 7 ): 1 – 96. Google Scholar 97. [doi.org]

• Dizziness

  Nonspecific prodromal symptoms (headache, dizziness) frequently (80%) precede livedo racemosa for 3.5 and (multi)focal neurological symptoms of fully developed disease for 9 years followed by progressive cognitive impairment (60%) 10 years later. [ncbi.nlm.nih.gov]

  In the natural course of patients with SS, neurological events such as headache (62%) and dizziness (54%) have been recorded. [elsevier.es]

  Neurological symptoms may include transient ischemic attacks (mini-strokes) and strokes; headache; and dizziness. [rareguru.com]

  Non-specific prodromal symptoms (headache, dizziness) often precede livedo reticularis by several years and this is followed by the development of focal neurological symptoms and then progressive cognitive impairment. [skin-disorders.net]

IgM and IgG, anti-beta-2-glycoprotein antibodies IgM and IgG) (3) thorough workup for conditions associated with hypercoagulability (4) echocardiography of the heart References: Schellong SM, Weissenborn K, et al. [meducator3.net]

Skin biopsy was nonspecific.1 Extensive immunologic, hematologic, and infectious workup was unremarkable.2 The patient was diagnosed with Sneddon syndrome and discharged on aspirin. [n.neurology.org]

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]

"Goodpasture Syndrome Workup". Medscape Reference. WebMD. Retrieved 14 March 2014. ^ a b c d e Kathuria, P; Sanghera, P; Stevenson, FT; Sharma, S; Lederer, E; Lohr, JW; Talavera, F; Verrelli, M (21 May 2013). Batuman, C (ed.). [en.wikipedia.org]

moderate (4-5, 4% risk), high (6-7, 8% risk) stroke in 48hrs MRI showing DWI lesion increases risk of re-stroke, if also vessel occlusion then 30% risk of restroke EXPRESS trial – 2007 ( UK ) Historical control compared to last few years of rapid workup [emilytam.com]

The success of warfarin in the treatment of antiphospholipid syndromes and the failure of immunosuppression and aspirin in the treatment of Sneddon's syndrome argue that warfarin anticoagulation may be the most appropriate intervention currently available [ncbi.nlm.nih.gov]

Management and treatment The most widely accepted treatment is anticoagulation with warfarin. [orpha.net]

In some cases, treatment has been started with intravenous immunoglobulins in patients with livedoid vasculopathy, with adequate outcomes.5 In our case, anticoagulant and antiproteinuric treatment was administered, with stability of renal function and [revistanefrologia.com]

While sensorimotor deficits after stroke in these patients had a good prognosis, neuropsychological deficits persisted. Of the 21 patients, 14 were incapable of gainful employment, 10 because of severe cognitive dysfunction. [ncbi.nlm.nih.gov]

The objectives were to assess the clinicoradiological pattern of SS without APL (SSAPL−) and its midterm prognosis. [onlinelibrary.wiley.com]

Prognosis Some symptoms tend to resolve over a very variable time course. Prevention of further vascular events with anticoagulation is the key to a good prognosis. [patient.info]

Sneddon’s syndrome patients have a worse prognosis and may represent a subgroup of patients who demands more rigorous follow-up. [journal.einstein.br]

Sneddon syndrome - a cerebral arteriopathy of unknown etiology, characterized by noninflammatory intimal hyperplasia of medium-size vessels. [medical-dictionary.thefreedictionary.com]

Sneddon's syndrome is a peculiar clinicopathological condition, probably with several etiologies, but is distinct from primary antiphospholipid syndrome. [ncbi.nlm.nih.gov]

Future therapy should identify the diverse treatment modalities for different etiological subgroups. [elsevier.es]

Summary Epidemiology SS has an estimated annual incidence of approximately 1/250,000. The disease predominantly affects women in young adulthood. [orpha.net]

Epidemiology Incidence is estimated at four cases per million population per year, but the true incidence may be higher because some patients are not diagnosed. [patient.info]

Epidemiology[edit] Sneddon's syndrome is a rare condition that is usually misdiagnosed.[3] It occurs in families and may be inherited in an autosomal dominant fashion. [en.wikipedia.org]

We suggest that these antibodies may be pathophysiologically related to the clinical manifestation observed in some patients with this syndrome. [ncbi.nlm.nih.gov]

Etiology This disease has at least two distinct etiologies, listed in the next section (pathophysiology). [dermatologyadvisor.com]

Gain fresh perspectives and up-to-date insights from the world’s leading authorities on the pathophysiology, diagnosis, and management of stroke. [books.google.de]

Prevention of coronary heart disease with pravastatin in men with hypercholesterolemia. ‎ [books.google.de]

Aspirin was not effective in preventing thrombosis. After the onset of oral anticoagulant therapy, no recurrences were seen. [ncbi.nlm.nih.gov]