Nephrogenic fibrosing dermopathy: A unique fibrosing skin condition of renal insufficiency associated with CD34/procollagen dual positive circulating fibrocytes

Abstract

Rationale Nephrogenic fibrosing dermopathy (NFD) is a recently described fibrosing skin condition with only about 100 cases reported between USA and Europe. Methods We report a case of a 52-year old kidney transplant patient who developed NFD three weeks after the onset of acute renal insufficiency. NFD is characterized by erythematous, brownish coalescent plaques that manifest as “woody” skin. The lesions may be itchy, painful, typically symmetrical with a predilection for extremities. Results The skin biopsy, typical for NFD, showed increased fibroblast-like cells in the dermis, some myofibroblasts, thickened collagen bundles separated by small clefts, and minimal inflammatory infiltrate. Systemic sclerosis was excluded clinically and histologically. The patient partially improved with oral corticosteroids and topical vitamin D. Hemodialysis was initiated. He is being evaluated for another kidney transplantation. The pathophysiology of NFD is incompletely understood, the histopathology suggests that fibrogenic cytokines like transforming growth factor-β1 may be involved in the disease. Recent dual immunohistochemical studies have shown that a unique cell known as circulating fibrocyte (CF) produces the characteristic histologic findings. It is unknown whether the CFs of NFD are aberrant in function, quantity, or merely stimulated by cytokines that are abnormally elevated in renal insufficiency. Conclusions To our knowledge, this is the first case of NFD outside Europe and USA. Since this is a new and rare condition, there is no standard treatment, but in almost all cases, resolution was associated with the return of normal kidney function. The collection and organization of information from NFD cases is crucial to the development of targeted therapeutic efforts.44