Catastrophic Antiphospholipid Syndrome: Skin, Eye and Brain Involvement

Purpose: To report on a patient with probable catastrophicantiphospholipid syndrome (CAPS) with eye, brain and skin involvement. Methods: Descriptive presentation of the case and of the relevant clinical photographs. Results: A 17-year-old girl presented with fatigue, loss of appetite, arthralgia, lower limb skin ulcers and livedo reticularis. Workup showed anemia, elevated ESR, CRP, and positive anti-phospholipid antibodies. Right eye funduscopy showed cotton-wool spots along the inferotemporal arcade with arteriolar occlusion, perivascular retinal hemorrhages, and diffuse retinal ischemia in the temporal peripheral retina. Fluorescein angiogram confirmed the widespread retinal ischemia. Brain MRI revealed several white matter lacunar infarcts, minute cortical/subcortical hemorrhages and subarachnoidal insular hemorrhage. The patient was treated with immunomodulatory therapy, Enoxaparin and retinal laser photocoagulation. Within 2 months of treatment, the facial livedo reticularis resolved and the leg ulcers markedly improved. Conclusion: CAPS causes multiple organ thrombosis and is associated with high rate of mortality.