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Arch Pathol Lab Med. 2006 Feb;130(2):209-12.

Multiorgan involvement in nephrogenic fibrosing dermopathy: an autopsy case and review of the literature.

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  • 1Department of Pathology, The Cleveland Clinic Foundation, Cleveland, Ohio 44195, USA.


Nephrogenic fibrosing dermopathy is a recently recognized, scleromyxedema-like fibrosing skin condition that occurs in individuals with acute or chronic renal failure. Although the early descriptions of this disorder describe a purely cutaneous disease process, 2 recent autopsy reports have identified apparent multiorgan fibrosis with involvement of skeletal muscle, myocardium, lungs, kidneys, and testes. We describe a 23-year-old man with nephrogenic fibrosing dermopathy and significant fibrosis of the atrial myocardium and dura mater, which was identified at autopsy. Dural fibrosis is a previously undescribed systemic manifestation of nephrogenic fibrosing dermopathy. The literature is reviewed.

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