Rare Diseases Symptoms Automatic Extraction

Köhlmeier-Degos's disease with primary intestinal manifestation.

[malignant atrophic papulosis]

Disseminated intestinal and cutaneous thromboangiitis (synonyms: Köhlmeier-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.

Diseases presenting "gastrointestinal tract" symptom

  • benign recurrent intrahepatic cholestasis
  • carcinoma of the gallbladder
  • cowden syndrome
  • cutaneous mastocytosis
  • dedifferentiated liposarcoma
  • epidermolysis bullosa simplex
  • erdheim-chester disease
  • esophageal adenocarcinoma
  • esophageal carcinoma
  • familial mediterranean fever
  • hirschsprung disease
  • malignant atrophic papulosis
  • megacystis-microcolon-intestinal hypoperistalsis syndrome
  • proteus syndrome
  • pyomyositis
  • triple a syndrome
  • waldenström macroglobulinemia
  • well-differentiated liposarcoma

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