title: 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.affiliation: Department of Dermatology, University Hospital, Zurich, Switzerland.
authors.forename: Severin
authors.initials: S
authors.lastname: Läuchli
authors.forename: Claudia
authors.initials: C
authors.lastname: Zortea-Caflisch
authors.forename: Frank O
authors.initials: FO
authors.lastname: Nestle
authors.forename: Günter
authors.initials: G
authors.lastname: Burg
authors.forename: Werner
authors.initials: W
authors.lastname: Kempf
mesh_terms: D000328:Adult; D005260:Female; D005355:Fibrosis; D005923:Glomerulosclerosis, Focal Segmental / Q000150:complications; D006801:Humans; D007676:Kidney Failure, Chronic / Q000150:complications*; D017893:Photopheresis; D012871:Skin Diseases / Q000209:etiology / Q000473:pathology* / Q000628:therapy*