A 61 -year-old man developed chest pain and intermittent claudication three months before his first visit. He complained of a pain of the lower extremities and difficulty in walking. The physical examination revealed portwine stain-like erythema demonstrating a geographic pattern with a necrotic center and pinpoint purpuras around a lesion on his right thigh. Arteriography revealed a complete occlusion of the abdominal artery at proximal-portion of the inferior mesenteric artery. The histopathologic examination of the portwine stain-like lesion showed a capillary proliferation throughout the dermis. His legs had become necrotic. He died due to sepsis and multiorgan failure after an amputation of both his legs.
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