AKA idiopathic livedo reticularis with cerebrovascular accidents
Mechanism
- Endothelial dysfunction (often antiphospholipid mediated) → arterial occlusion
- In skin (livedo racemosa) and brain
Clinical presentation
- Skin
- Livedo reticularis and racemosa
- Brain
- Phase 1: headaches and vertigo (before livedo reticularis)
- Phase 2: recurrent TIAs and CVAs
- Phase 3: cognitive decline and dementia
DDx
- Subcorneal pustular dermatosis type of IgA Pemphigus
Treatment
- Antiplatelet/antithrombotic agents
- Aspirin, clopidogrel
- Warfarin
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