Kohlmeier-Degos disease: a multisystem vasculopathy with progressive cerebral infarction

Kohlmeier-Degos disease, or malignant atrophic papulosis, is a rare and clinically distinctive vasculopathy characterised by cutaneous features with frequent gastrointestinal and neurological involvement and, almost invariably, with fatal outcome. The case is reported of a 22-year-old male who died of progressive cerebral infarction nine months from the time of development of pathognomonic skin lesions. Treatments with anti-platelet agents and plasma exchange were ineffectual in altering the progressive course of the disease. At autopsy, there was an extensive small vessel occlusive vasculopathy with similar features in skin, brain and bowel wall. The aetiology of Kohlmeier-Degos disease remains unknown. Diagnosis is on clinico-pathological grounds. Awareness of the disease is important, particularly in the differential diagnosis of cerebrovascular-arteritic-syndromes presenting in young adults.