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Renal Transplantation Could Reverse Dialysis-Associated Porphyria

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 patie...

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Autores principales: Barua, Sasanka k, Patel, Sachinkumar, Sarma, Debanga, Phukan, Mandeep, Bagchi, Puskal k
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9512316/
https://www.ncbi.nlm.nih.gov/pubmed/36176837
http://dx.doi.org/10.7759/cureus.28482
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author Barua, Sasanka k
Patel, Sachinkumar
Sarma, Debanga
Phukan, Mandeep
Bagchi, Puskal k
author_facet Barua, Sasanka k
Patel, Sachinkumar
Sarma, Debanga
Phukan, Mandeep
Bagchi, Puskal k
author_sort Barua, Sasanka k
collection PubMed
description 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.
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spelling pubmed-95123162022-09-28 Renal Transplantation Could Reverse Dialysis-Associated Porphyria Barua, Sasanka k Patel, Sachinkumar Sarma, Debanga Phukan, Mandeep Bagchi, Puskal k Cureus Urology 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. Cureus 2022-08-27 /pmc/articles/PMC9512316/ /pubmed/36176837 http://dx.doi.org/10.7759/cureus.28482 Text en Copyright © 2022, Barua et al. https://creativecommons.org/licenses/by/3.0/This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Urology
Barua, Sasanka k
Patel, Sachinkumar
Sarma, Debanga
Phukan, Mandeep
Bagchi, Puskal k
Renal Transplantation Could Reverse Dialysis-Associated Porphyria
title Renal Transplantation Could Reverse Dialysis-Associated Porphyria
title_full Renal Transplantation Could Reverse Dialysis-Associated Porphyria
title_fullStr Renal Transplantation Could Reverse Dialysis-Associated Porphyria
title_full_unstemmed Renal Transplantation Could Reverse Dialysis-Associated Porphyria
title_short Renal Transplantation Could Reverse Dialysis-Associated Porphyria
title_sort renal transplantation could reverse dialysis-associated porphyria
topic Urology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9512316/
https://www.ncbi.nlm.nih.gov/pubmed/36176837
http://dx.doi.org/10.7759/cureus.28482
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