Case of disseminated intestinal and cutaneous thromboangiitis (Kohlmeier-Degos' syndrome)
Gasanenko, L.N.; Kapust'ian, O.V.; Ovsiannikov, N.V.; Mazhbich, S.M.; Kapralov, E.A.; Bagisheva, N.V.; Kononov, A.V.; Kochetov, A.M.; Borisenko, M.V.
Terapevticheskii Arkhiv 82(2): 54-56
2010
ISSN/ISBN: 0040-3660 PMID: 20387678 Document Number: 642004
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).