Extracorporeal photopheresis improves nephrogenic fibrosing dermopathy/nephrogenic systemic fibrosis: Three case reports and review of literature

Abstract

Nephrogenic fibrosing dermopathy/nephrogenic systemic fibrosis (NFD/NSF) is a recently recognized systemic fibrosing disorder that develops in the context of renal insufficiency. Its predominant manifestation is severe cutaneous fibrosis, often causing disabling contractures of neighboring joints. No therapy is consistently effective, and etiology and pathogenesis remain obscure. However, gadolinium-containing contrast agents used in magnetic resonance imaging or angiography have recently been strongly linked to the development of fibrosis in NSF. We report a clinical response to treatment with extracorporeal photopheresis (ECP) using the Therakos UVAR XTS system in three cases of NFD/NSF associated with flexion contractures affecting all four limbs. All three patients were receiving renal replacement therapy for longstanding end-stage renal failure. All three showed clinical response with softening of skin plaques at the end of four cycles, and improved range of movement in all four limbs on completion of treatment. The first patient, with NFD/NSF of 4.5 year's duration, was able to resume most activities of daily living although still chairbound, while a second patient with more recent onset of NSF was able to walk short distances with the help of Zimmer frames by the end of 16-18 cycles of treatment, having been chairbound pretreatment. A third patient with milder initial symptoms also experienced significant improvement after a shorter course of ECP treatment. Our experience with these three patients confirms previous case reports, suggesting that ECP may be effective in NFD/NSF.