Nephrogenic fibrosing dermopathy and calciphylaxis with pseudoxanthoma elasticum-like changes


  • The data contained in this manuscript was presented at the 62nd Annual Meeting of the American Academy of Dermatology, Gross and Microscopic Dermatology Symposium on February 7, 2004.

Leslie Robinson-Bostom, MD, Department of Dermatology, Brown Medical School, Rhode Island Hospital-APC 10, 593 Eddy Street, Providence, RI 02903, USA
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Abstract:  Nephrogenic fibrosing dermopathy (NFD) and calciphylaxis are rare conditions that are associated with chronic kidney disease. Histopathologic changes, including dystrophic dermal calcification, often in association with elastic fibers have been observed in NFD and calciphylaxis. A pattern of dermal elastic fiber calcification that mimics pseudoxanthoma elasticum (PXE) has been previously reported as an incidental finding in the setting of calciphylaxis. Despite a shared association with renal disease and abnormal calcium deposits, however, NFD and calciphylaxis are discrete pathologic processes with distinct clinical and histopathologic features. Criteria for each are reviewed through case presentation of a patient meeting the clinical and histopathologic criteria for both NFD and calciphylaxis with histologic features mimicking PXE.