The Mystery of Sneddon Syndrome: Relationship with Antiphospholipid Syndrome and Systemic Lupus Erythematosus

Françès, Camille; Piette, Jean‐Charles

doi:10.1006/jaut.2000.0418

Cited by 100 publications

(68 citation statements)

References 41 publications

(48 reference statements)

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“…Because no specific test for SNS exists, clinical differentiation from other phenomenologically similar disorders may be difficult and has raised controversy [2, 11, 12, 13, 14, 15]. Various neurological problems have been associated with SNS and aPLs, including cerebrovascular events, chorea, seizures, myelopathy, atypical migraine-like events, acute encephalopathy, and dementia [3, 4, 12, 16, 17].…”

Section: Discussionmentioning

confidence: 99%

“…Varying findings on microscopy might be due to different underlying disease processes in patients clinically diagnosed with SNS [2]. It might also be due to sampling in different stages of the disease [23].…”

Section: Discussionmentioning

confidence: 99%

“…Approximately half of the patients report of headache. Cranial magnetic resonance imaging (MRI) detects cortical, subcortical or cortico-subcortical abnormalities suggestive of arterial ischemic infarcts which are frequently multiple [2, 4]. Usually, periventricular white matter changes are observed [5].…”

Section: Introductionmentioning

confidence: 99%

“…Patients with SNS and aPL are more likely to develop epilepsy (30% in aPL-positive vs. 10% in aPL-negative cases [2]). Optimal management of patients with SNS remains an unsolved problem.…”

Section: Introductionmentioning

confidence: 99%

“…The presence of several other organ manifestations such as cardiac and kidney lesions suggests that it is a systemic syndrome [2]. …”

Section: Introductionmentioning

confidence: 99%

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Therapy of Sneddon Syndrome

et al. 2002

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We report the case of a young woman with progressive cognitive decline and epilepsy. She showed ischemic cerebrovascular disease and proximal livedo racemosa. Antiphospholipid antibody (aPL) could not be detected and there were no microemboli on continuous transcranial Doppler ultrasonography monitoring. Histology of cerebral vessels showed intimal hyperplasia in small leptomeningeal venous vessels and micronecrosis of grey and white matter. We subsequently made the diagnosis of aPL-negative Sneddon Syndrome (SNS). Anticoagulation with warfarin could not be initiated because of a drug-resistant epilepsy with the risk of falls and subsequent bleeding; immunosuppression with steroids and azathioprine was ineffective, as was initial antiplatelet therapy with clopidogrel alone. However, when we intensified antiplatelet therapy by combining clopidogrel and ASS, a slowing of disease progression, as assessed by neuropsychological testing and magnetic resonance imaging, was noted on a follow-up after 6 months. Therapeutic options in SNS in both aPL-positive and aPL-negative patients with SNS are discussed.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

“…Patients with SNS and aPL are more likely to develop epilepsy (30% in aPL-positive vs. 10% in aPL-negative cases [2]). Optimal management of patients with SNS remains an unsolved problem.…”

Section: Introductionmentioning

confidence: 99%

“…The presence of several other organ manifestations such as cardiac and kidney lesions suggests that it is a systemic syndrome [2]. …”

Section: Introductionmentioning

confidence: 99%

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Therapy of Sneddon Syndrome

et al. 2002

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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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Rook's Textbook of Dermatology

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The Mystery of Sneddon Syndrome: Relationship with Antiphospholipid Syndrome and Systemic Lupus Erythematosus

Cited by 100 publications

References 41 publications

Therapy of Sneddon Syndrome

Therapy of Sneddon Syndrome

Generalized Net-like Erythema and Stroke in a Young Female

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