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

• Main Authors: Lyudmila Nikolaevna Gasanenko, Ol'ga Vladimirovna Kapust'yan, Nikolay Viktopovich Ovsyannikov, Sergey Mikhaylovich Mazhbich, Eduard Aleksandrovich Kapralov, Natal'ya Viktorovna Bagisheva, Aleksey Vladimirovich Kononov, Aleksandr Mikhaylovich Kochetov, Marina Viktorovna Borisenko, L N Gasanenko, O V Kapustyan, N V Ovsyannikov, S M Mazhbich, E A Kapralov, N V Bagisheva, A V Kononov, A M Kochetov, M V Borisenko
• Format: Article
• Language: Russian
• Published: "Consilium Medicum" Publishing house 2010-02-01
• Series: Терапевтический архив
• Subjects: thromboangiitis (kohlmeier-degos' syndrome); fatal intestinal and cutaneous syndrome; diagnosis; treatment
• Online Access: https://ter-arkhiv.ru/0040-3660/article/view/30552
• ISSN: 0040-3660; 2309-5342

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).