DEGOS-DISEASE - RADIOLOGICAL AND IMMUNOLOGICAL ASPECTS

We present an 18-year-old patient with Degos' disease who manifested t he sudden onset of paraplegia. Neurological examination revealed a rt- hemianopsia, paraparesis (with a sensory level at Th12), and a neuroge nic bladder. Brain MRI showed multiple cerebral infarctions accompanie d by small hemorrhagic areas and GdDTPA enhancement of the dura. A cer ebral angiogram displayed stenosis, ectasia, and aneurysms involving t he peripheral branch of arteries. Stenoses also were observed in the c eliac artery and small arteries in the lt-kidney. Skin biopsy exhibite d hyperkeratosis, atrophy of the epidermis, and necrobiosis of the col lagen layer. Laboratory examinations disclosed persistent elevations o f the thrombin-antithrombin III complex (TAT), plasmin-alpha 2 plasmin inhibitor complex (PIC), and cytotoxic T cell subset (CD8+CD11-), ill ustrating the coagulative, fibrinolytic, and immunological implication s of this disease.