Nephrogenic fibrosing dermopathy (NFD) is a scleroderma-like fibrosing disorder that develops in the setting of renal insufficiency. The first cases of NFD were identified in 1997. Since then, hundreds of cases have been identified. Most affected patients have been adults but several pediatric cases have now been reported. Although the cause of NFD is unknown, initial observations suggest that cells involved in wound repair and tissue remodeling may be aberrantly recruited in the absence of overt traumatic tissue injury.
Under a normal epidermis there is dense dermal fibrosis with a proliferation of fibroblasts which extends to the dermal-subcutaneous junction. There is a sparse perivascular lymphocytic infiltrate, and increased dermal mucin was demonstrated by colloidal iron stain.