SNEDDONS SYNDROME - A SYSTEMIC ARTERIO-OCCLUSIVE DISORDER

We describe a case of Sneddon's syndrome in a young woman with maligna nt hypertension and renal impairment. Kidney biopsy demonstrated intim al proliferation of small and medium-sized renal arteries similar to t hat seen in cutaneous arteries of patients with this syndrome, Ultrast ructural examination showed the proliferated intima to be composed of smooth muscle fibers, fibroblasts, monocytes, and extensive deposition of dense granular and light-staining amorphous materials. Our finding s support the proposition that Sneddon's syndrome may not be simply a neurocutaneous vascular disorder as originally described, but rather a systemic arterio-occlusive disorder with a variable clinical expressi on. (C) 1995 by the National Kidney Foundation, Inc.