Sneddon Syndrome: A Comprehensive Overview.

Area of Science:

• Neurology
• Dermatology
• Vascular Medicine

Background:

• Sneddon syndrome (SS) is a rare, progressive disorder characterized by livedo racemosa and recurrent cerebrovascular events.
• Histopathology reveals noninflammatory thrombotic vasculopathy in dermal and cerebral arteries.
• The exact etiopathogenesis remains unknown, with proposed mechanisms including autoimmune/inflammatory and thrombophilia factors.

Purpose of the Study:

• To summarize the clinical characteristics, proposed etiopathogenesis, and current treatment strategies for Sneddon syndrome.
• To highlight the diagnostic challenges and prognostic factors associated with SS.

Main Methods:

• Review of existing literature on Sneddon syndrome.
• Analysis of clinical presentations, histopathological findings, and treatment outcomes.

Main Results:

• SS predominantly affects women, with diagnosis typically around age 40, but childhood onset is reported.
• Neurological manifestations occur in phases: prodromal symptoms, recurrent strokes, and early-onset dementia.
• Livedo racemosa often precedes strokes by over a decade; antiplatelet/antithrombotic agents show promise in reducing stroke recurrence.

Conclusions:

• Sneddon syndrome presents with characteristic skin and neurological findings, often linked to thrombotic vasculopathy.
• While treatment is largely based on anecdotal evidence, antiplatelet/antithrombotic therapy appears beneficial for secondary stroke prevention.
• The neuropsychiatric prognosis is often poor, with significant cognitive deficits.