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Original Article

Köhlmeier-Degos's Disease with Primary Intestinal Manifestation

, , , &
Pages 1066-1070 | Received 10 Feb 1997, Accepted 23 Jun 1997, Published online: 08 Jul 2009
 

Abstract

Disseminated intestinal and cutaneous thromboangiitis (synonyms: Kohlmeier-Degos's syndrome, malignant atrophic papulosis, progressive arterial mesenterial vascular occlusive disease) is a rare, systemic vascular disease that is mainly manifested in the skin, gastrointestinal tract, and nervous system. The disease first appears as a necrotizing papulous dermatosis; as it generalizes, infarcted necroses develop in internal organs. These ischemic complications are the reason for the usually fatal outcome of the disease. A case report of a primary intestinal manifestation of this disease illustrates the clinical course, diagnosis, histopathologic findings, and differential diagnosis, with consideration of the current literature. Deposits of acid mucopolysaccharides and humoral immune mechanisms appear to play a role in the etiology and pathogenesis of this usually fatal vascular disease.

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