Scleromyxedema-like fibromucinosis in a patient undergoing hemodialysis

Fifteen hemodialysis patients were recently reported with a new scleromyxedema-like fibrosing disorder thought to be associated with renal dialysis. A case of this new clinicopathological entity is described as well as a limited review of the literature relevant to this disease. The patient was a 31-year-old Hispanic female with firm cutaneous plaques with brawny hyperpigmentation on the thighs and arms, which started 1 month after starting hemodialysis therapy for end-stage renal disease. There was no monoclonal gammopathy or evidence of systemic mucin deposition or other organ system disease. Histopathologically, the skin biopsy samples show haphazardly arranged dermal fibroblasts with associated collagen and small collections of interfibrillar mucin. The skin biopsy was interpreted as scleromyxedema-like fibromucinosis. This represents an additional case of this new renal dialysis-associated disorder. The disease may also occur in nondialysis patients, including those with liver disease.