Degos' disease: radiological and immunological aspects.
We present an 18-year-old patient with Degos' disease who manifested the sudden onset of paraplegia. Neurological examination revealed a rt-hemianopsia, paraparesis (with a sensory level at Th12), and a neurogenic bladder. Brain MRI showed multiple cerebral infarctions accompanied by small hemorrhagic areas and GdDTPA enhancement of the dura. A cerebral angiogram displayed stenosis, ectasia, and aneurysms involving the peripheral branch of arteries. Stenoses also were observed in the celiac artery and small arteries in the 1t-kidney. Skin biopsy exhibited hyperkeratosis, atrophy of the epidermis, and necrobiosis of the collagen layer. Laboratory examinations disclosed persistent elevations of the thrombin-antithrombin III complex (TAT), plasmin-alpha 2 plasmin inhibitor complex (PIC), and cytotoxic T cell subset (CD8+CD11-), illustrating the coagulative, fibrinolytic, and immunological implications of this disease.
Yoshikawa
H
H
Department of Neurology, Kanazawa University, School of Medicine, Ishikawa, Japan.
Maruta
T
T
Yokoji
H
H
Takamori
M
M
Yachie
A
A
Torii
Y
Y
D018784:Abdominal Abscess / Q000150:complications / Q000000981:diagnostic imaging / Q000276:immunology; D000293:Adolescent; D002544:Cerebral Infarction / Q000150:complications / Q000000981:diagnostic imaging / Q000276:immunology; D006801:Humans; D008297:Male; D010264:Paraplegia / Q000150:complications / Q000000981:diagnostic imaging / Q000276:immunology; D011859:Radiography; D012871:Skin Diseases / Q000150:complications / Q000000981:diagnostic imaging / Q000276:immunology; D001750:Urinary Bladder, Neurogenic / Q000150:complications / Q000000981:diagnostic imaging / Q000276:immunology