Nephrogenic fibrosing dermopathy treated with extracorporeal photopheresis.

Abstract

Nephrogenic fibrosing dermopathy (NFD) is a rare fibrosing skin disease of unknown etiology occurring in patients with terminal renal disease. It was first described in the year 2000. The histology of NFD shows an increased number of dendritic cells, fibroblasts and thickened collagen fibers resembling scleromyxedema. It can be distinguished from scleromyxedema by a different distribution pattern of the skin lesions with indurated plaques mainly on the extremities and the absence of paraproteinemia. As yet, no treatment for NFD has been proven to be uniformly efficient. We describe the case of a 40-year old patient with renal insufficiency who was treated with hemodialysis and who had undergone kidney transplantation. Two years after transplantation, she developed sclerodermiform brownish plaques on her extremities. The induration improved significantly after 4 cycles of extracorporeal photopheresis.

• Authors: Severin Läuchli, Claudia Zortea-Caflisch, Frank O Nestle, Günter Burg, Werner Kempf
• Journal: Dermatology (Basel, Switzerland) (Dermatology) Vol. 208 Issue 3 Pg. 278-80 ( 2004) ISSN: 1018-8665 [Print] Switzerland
• PMID: 15118390 (Publication Type: Case Reports, Journal Article)
• Copyright: Copyright 2004 S. Karger AG, Basel

Topics

• Adult
• Female
• Fibrosis
• Glomerulosclerosis, Focal Segmental (complications)
• Humans
• Kidney Failure, Chronic (complications)
• Photopheresis
• Skin Diseases (etiology, pathology, therapy)