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Köhlmeier-Degos syndrome

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A lethal cutaneovisceral syndrome characterized by the association of pathogonomic umbilicated porcelain-white skin papules due to vasculitis, followed by acute abdominal episodes, bowel perforation, peritonitis, sometimes with involvement of the nervous system and avascular patches in the conjunctivae. Both sexes affected but prevalent in males. Onset from middle age. Aetiology unknown. Histological findings show thrombosis of the blood vessels of the skin and intestinal mucosa and hyaline degeneration and necrobiosis of the cutaneous appendages.

First described by W. Köhlmeier in 1940. Robert Degos first recognized the disease as a distinct entity.


  • W. Köhlmeier:
    In: Frankfurter Zeitschrift für Pathologie, 1940, 54: 413. Multiple Hautnekrosen bei Thrombangiitis obliterans.
    Archiv für Dermatologie und Syphilis, Berlin, 1940-1941, 181: 783-792.
  • R. Degos, J. Delort, R. Tricot:
    Dermatite papulosquameuse atrophiante.
    Bulletin de la Société française de dermatologie et de syphiligraphie et de ses filiales, 1942, 49, 148-150.
  • R. Degos, J. Delort, R. Tricot:
    Papulose atrophiante maligne (syndrome cutanéo-intestinal mortel). Bulletins et memoires de la Société medicale des hôpitaux de Paris, 1948, 64: 803-806.

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