Nephrogenic fibrosing dermopathy: the first 6 years

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Abstract
Nephrogenic fibrosing dermopathy (NFD) is a newly recognized scleroderma-like fibrosing skin condition. It develops in patients with renal insufficiency. This review summarizes recent case reports and examines theories of disease pathogenesis. Information from the Yale University NFD Registry Project, as well as published case reports, is included to provide a contextual framework upon which to base these theories. Recent studies have contributed to a clearer definition of the clinical spectrum, epidemiology, and pathogenesis of NFD. The findings of yellow scleral plaques and circulating antiphospholipid antibodies have been proposed as markers of NFD in recent case reports. In addition, epidemiologic data have yielded several distinct clinical patterns of disease onset. Lastly, dual immunohistochemical staining for CD34 and procollagen in the spindle cells of NFD suggest that the dermal cells of NFD may represent circulating fibrocytes recruited to the dermis—a finding previously undescribed in normal skin. Scenario classification of NFD is a means for simplifying the search for multifactorial disease triggers, and may be helpful in predicting prognosis and response to therapy. The technique of dual immunolabeling, although not yet fully characterized as a diagnostic test, may provide a sensitive and specific method of diagnosis, as well as a starting point in the investigation of other cutaneous fibrosing disorders. The postulate that NFD may represent a systemic disorder mediated by aberrantly functioning circulating fibroblast precursor fibrocytes is explored.