Abstract
We report a case of suspected nephrogenic fibrosing dermopathy that developed during long-term hemodialysis. The patient was a 51-year-old female who had been on hemodialysis for end-stage renal disease due to glomerulonephritis since the age of 34. In 2002, she developed fever, polyarthralgia, dyspnea on exertion, and cardiac enlargement in association with waxy hardening skin over her lower extremities. The laboratory studies were positive for antinuclear antibody and rheumatoid factor, but were negative for anti Scl-70 antibody. There were no clinical data suggestive of malignancy. Biopsy of the sclerotic skin lesions of her leg demonstrated fibrosing lesions with thick collagen bundles and CD 34 positive dendritic cells without infiltration of eosinophils or other inflammatory cells. Although the etiology of her fever could not be determined, the patient became spontaneously afebrile after approximately 3 weeks. Her dyspnea improved in response to fluid restriction, but the sclerotic skin change remained progressive. In March 2003, the patient was admitted for swelling and redness of her left forearm bearing an arterio-venous fistula for hemodialysis associated with joint contracture. Stenosis of the venous outflow was suspected and surgical closure of the fistula resulted in prompt disappearance of the forearm swelling. However, cutaneous sclerosis of the legs, the forearms and the trunk persisted although there was no sclerotic cutaneous change on her face. Clinical presentation, laboratory data and findings of skin biopsy made it unlikely that the patient's cutaneous sclerosis was caused by systemic sclerosis, scleromyxedema, eosinophilic fasciitis, or amyloidosis, and the skin lesion was suspected to be nephrogenic fibrosing dermopathy (NFD).
