[Case of disseminated intestinal and cutaneous thromboangiitis (Kohlmeier-Degos' syndrome)]

Ter Arkh. 2010;82(2):54-6.
[Article in Russian]

Abstract

Disseminated intestinal and cutaneous thromboangiitis (Kohlmeier-Degos' syndrome) is a rare syndrome of unknown etiology, which is characterized by cutaneous and visceral involvement. Two (cutaneous and visceral) stages are identified. A fatal outcome is inevitable although there may be a long (8-20-year) course of the disease during a benign process. The causes of death are peritonitis, central nervous system impairments, pleurisy, pericarditis. The paper describes a case of disseminated intestinal and cutaneous thromboangiitis (Kohlmeier-Degos' syndrome) in a 32-year-old female patient who has died from intoxication due to severely impaired permeability of the epithelial barrier mainly of the large bowel (diphtheritic inflammation of the mucosa, severe inflammatory edema of the submucous layer, and fibrinous impregnation of the serous membrane).

Publication types

  • Case Reports
  • English Abstract

MeSH terms

  • Adult
  • Anti-Bacterial Agents / therapeutic use*
  • Diagnosis, Differential
  • Echocardiography
  • Endoscopy, Gastrointestinal
  • Erythrocyte Transfusion / methods*
  • Fatal Outcome
  • Female
  • Glucocorticoids / therapeutic use*
  • Humans
  • Laparoscopy
  • Malignant Atrophic Papulosis / diagnosis*
  • Malignant Atrophic Papulosis / therapy
  • Syndrome
  • Tomography, X-Ray Computed

Substances

  • Anti-Bacterial Agents
  • Glucocorticoids