Successful treatment of three cases of nephrogenic fibrosing dermopathy with extracorporeal photopheresis.

Details

Serval ID
serval:BIB_32A75593BD25
Type
Article: article from journal or magazin.
Publication sub-type
Case report (case report): feedback on an observation with a short commentary.
Collection
Publications
Title
Successful treatment of three cases of nephrogenic fibrosing dermopathy with extracorporeal photopheresis.
Journal
British Journal of Dermatology
Author(s)
Gilliet M., Cozzio A., Burg G., Nestle F.O.
ISSN
0007-0963 (Print)
ISSN-L
0007-0963
Publication state
Published
Issued date
2005
Volume
152
Number
3
Pages
531-536
Language
english
Notes
Publication types: Case Reports ; Journal ArticlePublication Status: ppublish
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.
OBJECTIVES: 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.
CONCLUSION: These data indicate that ECP may represent a valuable therapeutic option for NFD.
Keywords
Adult, Female, Fibrosis, Humans, Kidney Failure, Chronic/complications, Leg Dermatoses/etiology, Leg Dermatoses/therapy, Middle Aged, Photopheresis, Skin/pathology, Skin Diseases/etiology, Skin Diseases/pathology, Treatment Outcome
Pubmed
Web of science
Create date
19/11/2012 19:37
Last modification date
20/08/2019 14:18
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