title: Successful treatment of three cases of nephrogenic fibrosing dermopathy with extracorporeal photopheresis.
abstract: BACKGROUND
  Nephrogenic fibrosing dermopathy (NFD) is a recently described cutaneous fibrosing disorder associated with renal dysfunction. Patients present with thickened skin or oedematous skin with indurated papules and plaques involving extremities and trunk, and often associated with disabling contracture of the adjacent joints. The aetiology and pathogenesis remain largely unknown. As a consequence, therapeutic measures with proven efficacy are nonexistent to date.
  
  
  OBJECTIVE
  To consider treatment with extracorporal photopheresis (ECP) in three patients. Patients We report three new cases of NFD with the characteristic clinical and pathological features. Two patients required haemodialysis due to end-stage renal failure, despite prior renal transplantation. One patient had renal dysfunction but was never on dialysis, nor had she been transplanted. ECP treatment was administered at intervals of 2-4 weeks.
  
  
  RESULTS
  All three patients showed a softening of the skin lesions and a marked improvement of the joint motility starting after four cycles of ECP. One patient developed a complete regression of her skin lesions after 16 cycles of ECP, and response to therapy was observed despite constantly elevated renal values.
  
  
  CONCLUSIONS
  These data indicate that ECP may represent a valuable therapeutic option for NFD.
authors.affiliation: Department of Dermatology, Zurich University Hospital, Gloriastrasse 31, 8091 Zurich, Switzerland.
authors.forename: M
authors.initials: M
authors.lastname: Gilliet
authors.forename: A
authors.initials: A
authors.lastname: Cozzio
authors.forename: G
authors.initials: G
authors.lastname: Burg
authors.forename: F O
authors.initials: FO
authors.lastname: Nestle
mesh_terms: D000328:Adult; D005260:Female; D005355:Fibrosis; D006801:Humans; D007676:Kidney Failure, Chronic / Q000150:complications*; D007868:Leg Dermatoses / Q000209:etiology / Q000628:therapy; D008875:Middle Aged; D017893:Photopheresis; D012867:Skin / Q000473:pathology; D012871:Skin Diseases / Q000209:etiology / Q000473:pathology / Q000628:therapy*; D016896:Treatment Outcome