Sneddon's syndrome: a systemic arterio-occlusive disorder

We describe a case of Sneddon's syndrome in a young woman with malignant hypertension and renal impairment. Kidney biopsy demonstrated intimal proliferation of small and medium-sized renal arteries similar to that seen in cutaneous arteries of patients with this syndrome. Ultrastructural 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 findings support the proposition that Sneddon's syndrome may not be simply a neurocutaneous vascular disorder as originally described, but rather a systemic arterioocclusive disorder with a variable clinical expression.