KOHLMEIER-DEGOSS-DISEASE WITH PRIMARY INTESTINAL MANIFESTATION

Disseminated intestinal and cutaneous thromboangiitis (synonyms: Kohlm eier-Degos's syndrome, malignant atrophic papulosis, progressive arter ial mesenterial vascular occlusive disease) is a rare, systemic vascul ar disease that is mainly manifested in the skin, gastrointestinal tra ct, and nervous system. The disease first appears as a necrotizing pap ulous dermatosis; as it generalizes, infarcted necroses develop in int ernal organs. These ischemic complications are the reason for the usua lly fatal outcome of thr disease. A case report of a primary intestina l manifestation of this disease illustrates the clinical course, diagn osis, histopathologic Endings, and differential diagnosis, with consid eration of the current literature. Deposits of acid mucopolysaccharide s and humoral immune mechanisms appear to play a role in the etiology and pathogenesis of this usually fatal vascular disease.