Sneddon's Syndrome

Zelger, Bernhard

doi:10.1001/archderm.1993.01680250049006

Cited by 90 publications

(6 citation statements)

References 9 publications

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“…Sneddon syndrome is a rare vasculopathy (4 new cases per million per year) that most commonly occurs in women between the ages of 20 and 40 years . It is characterized by the combination of repeated strokes with widespread livedo racemosa.…”

Section: Discussionmentioning

confidence: 99%

Generalized Net-like Erythema and Stroke in a Young Female

2018

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A woman in her 40s presented with a 4-hour history of ataxia and left-sided hemidysesthesia. Annular patches of violaceous erythema forming an irregular net-like pattern were noted on her trunk and extremities (Figure , A and B). The patient stated that these skin changes had appeared 2 years prior. Her medical history was significant for 5 strokes over the past 6 years and Raynaud syndrome for the past 2 years. In the past, despite extensive workup, no definitive diagnosis had been established. Complete blood cell count, liver function tests, creatinine, C-reactive protein, erythrocyte sedimentation rate, and urinalysis results were normal. Antinuclear antibodies and complement C3 and C4 levels were unremarkable. Antiphospholipid antibody and lupus anticoagulant screening results were repeatedly negative and no evidence of thrombophilia was found. Magnetic resonance imaging showed acute dot-like diffusion anomalies in the medial and posterior cerebral artery-supplied areas, as well as old stroke residues (Figure , C). Transesophageal echocardiography showed no abnormalities. A skin biopsy sample was taken from the patient's left thigh (Figure , D). Clinical image A Clinical image B Magnetic resonance image C Histopathologic image D Figure. A, Patient's lower back and buttocks and, B, thighs and upper calves show generalized, irregular, violaceous, net-like erythema. C, Magnetic resonance imaging of the brain showing old stroke residues. D, Hematoxylin-eosin stain (original magnification ×10). WHAT IS YOUR DIAGNOSIS? A. Cutis marmorata telangiectatica congenita (van Lohuizen syndrome) B. Systemic lupus erythematosus C. Ataxia-telangiectasia (Louis-Bar syndrome) D. Sneddon syndrome Clinical Review & Education

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Section: Discussionmentioning

confidence: 99%

Generalized Net-like Erythema and Stroke in a Young Female

2018

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“…The pathophysiology of ischemic events in patients with SS is understood poorly 6. Analysis of skin biopsy specimens of the livedo reticularis suggests microvascular events based on an endothelial target as a primary event 9–13. It is speculated that a similar process may underlie vascular lesions in the central nervous system 9, 14.…”

Section: Discussionmentioning

confidence: 99%

“…Sudden motor deficit, aphasia, visual field defect and acute encephalopathy are frequent as the first neurological event. Other neurological signs described in the literature include migranous and nonmigranous headache, seizures, and chorea 5, 11. Global cognitive decline usually appears late in the course of the disease, due to multiple infarcts 12–15.…”

Section: Discussionmentioning

confidence: 99%

Tremor as the first neurological manifestation of Sneddon's syndrome

et al. 2004

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We report on a 54-year-old woman with Sneddon's syndrome manifested by livedo reticularis, fetal losses, hypertension, and high antinuclear antibody titres. At the age of 42 years she developed tremor of the trunk, limbs, and head only in the standing position that interfered with walking, followed some years later by cognitive decline and a parkinsonian syndrome. T2-weighted brain magnetic resonance imaging showed high signal in cortical areas, basal ganglia, midbrain, and cerebellum.

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“…Some studies suggest that the lesions occurring in the affected medium-sized vessels are inflammator y in nature. [8][9][10] Evidence supporting this hypothesis have included elevated erythrocyte sedimentation rates during acute events, complement consumption, immune complexes in circulation, and vascular inflammation in skin biopsy specimens. 11 Other studies and case reports have found no evidence of inflammation involving digital artery biopsies or vessels of skin biopsy.…”

Section: Commentmentioning

confidence: 99%

“…Hypertension, in particular, is associated with cerebrovascular disease pro-gression. 8 Sneddon syndrome has also been associated with cardiac valvulopathy and ischemic heart disease. 20,21 One retrospective study showed that the severity of MR images correlate with the severity of the disease.…”

Section: Commentmentioning

confidence: 99%