A case of nephrogenic fibrosing dermopathy

Abstract

Introduction: Nephrogenic fibrosing dermopathy is a recently recognised fibrosing disorder originally identified as a scleromyxoedema-like cutaneous disease in patients with renal disease. Clinical Picture: A 45-year-old lady with systemic lupus erythematosus presented with well-defined erythematous, non-tender, indurated plaques on both legs 4 months after haemodialysis for rapidly progressive glomerulonephritis and acute renal failure. Skin biopsy showed dermal fibrosis with increased proliferation of dermal fibroblasts and collagen bundles separated by clefts. There were increased dermal deposits of mucin and an increase in elastic fibres. Treatment and Outcome: Haemodialysis was instituted for 2 weeks followed by monthly intravenous cyclophosphamide. Skin lesions remained unchanged 8 months later despite normalisation of renal function. Conclusion: Nephrogenic fibrosing dermopathy appears to be a definite new entity in patients with underlying renal insufficiency. Further multi-centre collaborative study is necessary to identify the prevalence, cause, treatment and prognosis of this disorder.