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Weber-Christian disease associated with the tip variant of focal segmental glomerulosclerosis: a case report
Kevin A Sterling1, Sabine Karam, Nora Taylor
1Division of Kidney Disease and Hypertension, George Washington University School of Medicine, Washington, DC 20037, USA. sterling_kevin@hotmail.com
Abstract:
Weber-Christian disease (W-CD) is associated with relapsing nodular panniculitis and a variety of systemic findings. Renal parenchymal involvement has been rarely reported. The authors describe a case of nephrotic syndrome in an African-American man with a W-CD flare. The patient had chills and low-grade fever with painful lower extremity skin lesions. A renal biopsy demonstrated the tip variant of focal segmental glomerulosclerosis (FSGS). The kidney biopsy also suggested parenchymal involvement by W-CD disease, with supportive ultrastructural findings. The synchronous W-CD flare and biopsy-proven FSGS and the rapid and sustained response of both to limited treatment suggest a causative association.
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