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

[malignant atrophic papulosis]

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 .

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Diseases presenting "is a rare and clinically distinctive vasculopathy characterised by cutaneous features with frequent gastrointestinal and neurological involvement and" symptom

malignant atrophic papulosis

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