Treatment of Skin Ulcers Secondary to Sneddon Syndrome With Alprostadil (Prostaglandin E1)

Report of a Case | A woman in her 50s with a history of SS of 20 years’ evolution came to the dermatology department with ulceration on both legs. The patient had a history of lacunar infarction and was taking acetylsalicylic acid for prevention of cerebral ischemic events. Skin ulcers were previously treated with systemic corticosteroids and clopidogrel with no response. Physical examination revealed the presence of ulcerations of an irregular shape 1 to 5 cm in diameter with retiform purpura in both lower limbs (Figure, A). In addition, racemous livedo was observed on almost all the body surface. Peripheral pulses in upper and lower limbs were preserved bilaterally. No signs of severe chronic venous insufficiency or palpable purpura were observed. Laboratory findings for differential diagnosis of SS were normal or negative. Dermatopathologic analysis showed presence of fibrin thrombi and wall thickening in the papillary dermis with neovascularization phenomena. No fibrinoid necrosis of the vessel wall, neutrophilic infiltrate, or leukocytoclasia was observed. A diagnosis of SS was confirmed. Treatment with intravenous alprostadil (prostaglandin E1 [PGE-1]) (Prostavasin; Gebro Pharma GmbH) was started at doses of 60 μg every 24 hours for 5 days and then a dose of 60 μg every 24 hours monthly as maintenance. From the first infusion, the patient showed rapid improvement in pain. After 3 months of treatment, complete healing of the skin ulcers was observed (Figure, B). At last follow-up, the patient had been treated for 6 months with a monthly infusion of alprostadil and remained asymptomatic. No adverse effects were observed.