Nephrogenic fibrosing dermopathy: A series in a non-Western population

Abstract

Nephrogenic fibrosing dermopathy (NFD) is a newly proposed clinical entity resembling scleromyxedema. This is mainly found in patients with chronic renal failure who have undergone hemodialysis. Nearly all cases have been reported in the Western population. We sought to assess whether NFD cases occurred in a non-Western population and to ascertain the frequency, clinical characteristics, histopathology, and natural history of the condition in the event of its occurrence. Patients postdialysis in the nephrology department of a tertiary referral hospital in Kolkata, India, were evaluated clinically and histopathologic assessment was done on the basis of a clinical scoring to ascertain the frequency of the condition and its characteristics. This report is based on the findings of the first 3 years of an ongoing study. NFD was diagnosed in 6 of 2146 patients postdialysis 0.28%). In transplant recipients undergoing dialysis, NFD was diagnosed in 2 of 43 patients (4.65%). The average number of dialyses undergone by the patients was 7.2 (range: 2-16). Of the 6 cases of NFD, 5 conformed to the diagnosis of chronic kidney disease-V. Among them, 3 were noninsulin-dependent diabetes mellitus and one each were cases of chronic glomerulonephritis and systemic lupus erythematosus. One case followed dialysis in acute tubular necrosis. All cases clinically showed circumscribed thickening, roughened texture, hardening, xerosis, and infiltrated papules, plaques, or both. One case showed rippled pigmentation, four showed varied hyperpigmentation, two demonstrated localized fibrosis, and one resembled papular mucinosis. Increased thickness of dermis, increased number of plump spindle cells, abundance of thick collagen bundles with prominent clefts, and sparse small, multinucleated histiocytes were the hallmark of microscopic findings. The inherent nature of the study precluded the inclusion of NFD cases without any background of dialysis, which have now been reported. No statistical conclusions may be established from our small series. To our knowledge, this is the first reported series of NFD outside North America and Europe.