Literature DB >> 36176837

Renal Transplantation Could Reverse Dialysis-Associated Porphyria.

Sasanka K Barua1, Sachinkumar Patel1, Debanga Sarma1, Mandeep Phukan1, Puskal K Bagchi1.   

Abstract

Pseudoporphyria (PP) is a relatively infrequent, photodistributed bullous dermatosis that clinically, histopathologically, and immunologically resembles porphyria cutanea tarda (PCT), but is not accompanied by porphyrin abnormalities in the serum, urine, or stool. It was initially described in patients with renal failure on dialysis as 'bullous dermatosis of hemodialysis.' Pseudoporphyria has been seen in patients with end-stage renal disease on hemodialysis. No treatment has proved efficacious in the management of pseudoporphyria. However, N-acetylcysteine has been anecdotally reported to be effective in the management of hemodialysis-related pseudoporphyria and other porphyric diseases. Our patient had developed multiple skin lesions all over the body when hemodialysis started. The lesions were erythematous with fluid-filled vesicles, and bullae with cutaneous fragility that were evaluated and diagnosed as pseudoporphyria. The patient was treated with available all medication in the literature but was not relieved. However, all skin lesions completely healed within 22 days post renal transplantation. Renal transplantation proved to be the cure for dialysis-induced pseudoporphyria resistant to conventional drug therapy.
Copyright © 2022, Barua et al.

Entities:  

Keywords:  hemodialysis; n-acetylcysteine; porphyria cutanea tarda; porphyrin profile; pseudoporphyria; renal transplantation

Year:  2022        PMID: 36176837      PMCID: PMC9512316          DOI: 10.7759/cureus.28482

Source DB:  PubMed          Journal:  Cureus        ISSN: 2168-8184


  11 in total

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Authors:  J Vadoud-Seyedi; G de Dobbeleer; T Simonart
Journal:  Br J Dermatol       Date:  2000-03       Impact factor: 9.302

Review 2.  Pseudoporphyria.

Authors:  J J Green; S M Manders
Journal:  J Am Acad Dermatol       Date:  2001-01       Impact factor: 11.527

3.  Successful outcome of haemodialysis-induced pseudoporphyria after short-term oral N-acetylcysteine and switch to high-flux technique dialysis.

Authors:  Cesare Massone; Christina M Ambros-Rudolph; Alessandro Di Stefani; Robert R Müllegger
Journal:  Acta Derm Venereol       Date:  2006       Impact factor: 4.437

4.  Porphyria cutanea tarda in a human immunodeficiency virus-infected patient: treatment with N-acetyl-cysteine.

Authors:  H Binet; T Simonart; J P Van Vooren; M Heenen; C Liesnard; M L Delforge; C M Farber; D Parent
Journal:  Int J Dermatol       Date:  1998-09       Impact factor: 2.736

5.  Jan Gösta Waldenström and rheumatology.

Authors:  Frank A Wollheim
Journal:  Ann Rheum Dis       Date:  2019-01-12       Impact factor: 19.103

Review 6.  Porphyria.

Authors:  D Montgomery Bissell; Karl E Anderson; Herbert L Bonkovsky
Journal:  N Engl J Med       Date:  2017-08-31       Impact factor: 91.245

7.  Cysteine ameliorates photosensitivity in erythropoietic protoporphyria.

Authors:  J E Roberts; M Mathews-Roth
Journal:  Arch Dermatol       Date:  1993-10

Review 8.  Porphyrias.

Authors:  Hervé Puy; Laurent Gouya; Jean-Charles Deybach
Journal:  Lancet       Date:  2010-03-13       Impact factor: 79.321

Review 9.  Dermatologic conditions seen in end-stage renal disease.

Authors:  Katharine B Cordova; Thomas J Oberg; Mohsin Malik; Leslie Robinson-Bostom
Journal:  Semin Dial       Date:  2009 Jan-Feb       Impact factor: 3.455

10.  Pseudoporphyria associated with nonhemodialyzed renal insufficiency, successfully treated with oral N-acetylcysteine.

Authors:  A C Katoulis; D Ferra; E Toumbis; E Papadavid; A Kanelleas; I Panayiotides; D Rigopoulos
Journal:  Case Rep Dermatol Med       Date:  2013-04-10
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