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skos:definition |
MSH: Variously described as a vasculopathy, endovasculitis, or occlusive arteriopathy, this condition occurs in a benign cutaneous form and a lethal multiorgan systemic variant. It is characterized by a narrowing and occlusion of the lumen of small to medium-sized blood vessels, leading to ischemia and infarction in the involved organ systems. The etiology and pathophysiology are unknown.,NCI: A rare disorder of unknown etiology. It may present as a cutaneous disorder with a benign clinical course or a systemic disorder with a grim prognosis. Both the cutaneous and systemic variants are characterized by the presence of papular skin lesions. The systemic variant, in addition to the skin papules, is characterized by gastrointestinal manifestations including abdominal pain and distention, nausea, vomiting, diarrhea, intestinal infarction, and intestinal perforation. The central and peripheral nervous systems may also be involved in the systemic variant.
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