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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 rthemianopsia, 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 lt‐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‐α2 plasmin inhibitor complex (PIC), and cytotoxic T cell subset (CD8 + CD11‐), illustrating the coagulative, fibrinolytic, and immunological implications of this disease.