Nephrogenic Fibrosing Dermopathy

Abstract

Nephrogenic fibrosing dermopathy is a rare, recently described fibrotic skin condition that primarily affects patients with a history of renal disease.We report 2 cases of nephrogenic fibrosing dermopathy and review clinical and histopathologic features with differential diagnosis and more.Case #1 was a 64‐year‐old black male with chronic renal insufficiency which progressed to end stage renal disease requiring hemodialysis. After two days, he developed blisters on both his hands. By a month, his blister lesions resolved leaving scaly skin with some skin thickening and tightening in the hands and forearms. Over the course of one month, skin over above‐mentioned areas began to harden and stiffen. His fingers began to coalesce and contract resulting in fixation of hand grip. He also developed flexing contracts at the elbows bilaterally. Skin biopsy revealed the diagnosis of nephrogenic fibrosing dermopathy with giant cells reaction. Case #2 was a 44‐year‐old black male with a hx of nephropathy. He eventually developed end stage renal disease requiring hemodialysis. One month after, he was found to have thickened, sclerotic appearing skin involving the upper back, hands, and forearms with flexing contracts of the all the small joints. Histopathology was most consistent with nephrogenic fibrosing dermopathy.