A COMBINATION OF LIVEDO RACEMOSA, OCCLUSION OF CEREBRAL BLOOD-VESSELS, AND NEPHROPATHY - KIDNEY INVOLVEMENT IN SNEDDONS SYNDROME

A 59-year-old woman with retinal vein thrombosis and livedo racemosa h ad hematuria (4+) and proteinuria (1.7 g/day). Skin biopsy showed swol len blood vessel walls with infiltration of mononuclear cells, which w ere compatible with livedo racemosa (vasculitis). Magnetic resonance i maging (MRI) of the brain demonstrated multiple lacunar infarctions in the basal ganglia and white matter. Renal biopsy showed that small ro und cells had infiltrated into the interstitium, and a reticular struc ture was observed in the glomerular hilus. An amorphous substance comp osed of a single cell was present in the glomerular capillary lumen, I mmunofluorescent study demonstrated the deposition of only IgA, in a s egmental pattern differing from the diffuse global mesangial pattern s een in IgA nephropathy. After combined therapy including 40 mg/day pre dnisolone, 50 mg/day cyclophosphamide, antiplatelet drug, and anticoag ulant was started, proteinuria and hematuria improved to 0.5 g/day and 2+, respectively, at the time of discharge, Sneddon's syndrome is a r are entity characterized by livedo racemosa and cerebrovascular lesion s. In our patient with livedo racemosa, occlusion of cerebral blood ve ssels, and nephropathy with segmental immunoglobulin A (IgA) depositio n, no antiphospholipid antibodies were detected on routine examination , Dermatologists, neurologists, psychiatrists, and nephrologists shoul d be aware of the existence of Sneddon's syndrome with nephropathy (LI -O-N). (C) 1995 by the National Kidney Foundation, Inc.