Literature DB >> 24718503

[Porphyria cutanea tarda with sclerodermatous changes and hemochromatosis].

E Wallaeys1, U Thierling, E Lang, N J Neumann, J Frank.   

Abstract

A 55-year-old woman presented with blistering on the back of her hands and shiny, thickened skin in her décolletage. Laboratory examination revealed increased urinary total and high carboxylated porphyrins and homozygosity for mutation C282Y in the HFE gene. Histopathology showed thickened collagen fibers in the presternal region. Based on these findings we made the diagnosis of porphyria cutanea tarda with pseudoscleroderma and hemochromatosis. Pseudoscleroderma is a rare complication of PCT but can also constitute the first cutaneous symptom of the disease, leading the way to diagnosis. Usually, adequate treatment of PCT with normalization of porphyrin values also results in improvement of pseudoscleroderma.

Entities:  

Mesh:

Substances:

Year:  2014        PMID: 24718503     DOI: 10.1007/s00105-014-2783-6

Source DB:  PubMed          Journal:  Hautarzt        ISSN: 0017-8470            Impact factor:   0.751


  6 in total

Review 1.  Therapy of porphyria cutanea tarda.

Authors:  Erich Köstler; Uwe Wollina
Journal:  Expert Opin Pharmacother       Date:  2005-03       Impact factor: 3.889

Review 2.  Scleroderma and pseudo-scleroderma: uncommon presentations.

Authors:  Uwe-Frithjof Haustein
Journal:  Clin Dermatol       Date:  2005 Sep-Oct       Impact factor: 3.541

Review 3.  Porphyria cutanea tarda--when skin meets liver.

Authors:  Jorge Frank; Pamela Poblete-Gutiérrez
Journal:  Best Pract Res Clin Gastroenterol       Date:  2010-10       Impact factor: 3.043

4.  [Sclerodermatous changes in porphyria cutanea tarda: six cases].

Authors:  R Khayat; A Dupuy; I Pansé; M Bagot; F Cordoliani
Journal:  Ann Dermatol Venereol       Date:  2013-05-31       Impact factor: 0.777

5.  Low-dose hydroxychloroquine is as effective as phlebotomy in treatment of patients with porphyria cutanea tarda.

Authors:  Ashwani K Singal; Csilla Kormos-Hallberg; Chul Lee; Vaithamanithi M Sadagoparamanujam; James J Grady; Daniel H Freeman; Karl E Anderson
Journal:  Clin Gastroenterol Hepatol       Date:  2012-09-14       Impact factor: 11.382

6.  Sclerodermatous changes of face, neck and scalp associated with familial porphyria cutanea tarda.

Authors:  C L Thomas; M N Badminton; J R S Rendall; A V Anstey
Journal:  Clin Exp Dermatol       Date:  2008-05-21       Impact factor: 3.470

  6 in total
  1 in total

Review 1.  Cutaneous Manifestations of Scleroderma and Scleroderma-Like Disorders: a Comprehensive Review.

Authors:  Caterina Ferreli; Giulia Gasparini; Aurora Parodi; Emanuele Cozzani; Franco Rongioletti; Laura Atzori
Journal:  Clin Rev Allergy Immunol       Date:  2017-12       Impact factor: 8.667

  1 in total

北京卡尤迪生物科技股份有限公司 © 2022-2023.