Successful Treatment of a Widespread Pemphigus Chronicus Familiaris (Hailey-Hailey) By Erbium-YAG-Laser

BACKGROUND: Familial chronic pemphigus or Hailey-Hailey disease (OMIM 169600) is a rare, autosomal dominant blistering skin disorder the genetic background are mutations of the ATP2 C1 gene. The treatment is challenging. CASE REPORT: A 48-year-old Caucasian female patient presented to the department...

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Autores principales: Wollina, Uwe, Hansel, Gesina, Lotti, Torello, Vojvodic, Aleksandra
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Republic of Macedonia 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6910780/
https://www.ncbi.nlm.nih.gov/pubmed/31850125
http://dx.doi.org/10.3889/oamjms.2019.764
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author Wollina, Uwe
Hansel, Gesina
Lotti, Torello
Vojvodic, Aleksandra
author_facet Wollina, Uwe
Hansel, Gesina
Lotti, Torello
Vojvodic, Aleksandra
author_sort Wollina, Uwe
collection PubMed
description BACKGROUND: Familial chronic pemphigus or Hailey-Hailey disease (OMIM 169600) is a rare, autosomal dominant blistering skin disorder the genetic background are mutations of the ATP2 C1 gene. The treatment is challenging. CASE REPORT: A 48-year-old Caucasian female patient presented to the department with a relapse of her Pemphigus chronicus familiaris (Hailey-Hailey). No other medical diseases were known. On examination, we observed an otherwise healthy woman with widespread erosive lesions on the neck, axillae, groins, submammary fold and anal fold. She reported burning sensations and an unpleasant odour. The diagnosis had been confirmed earlier by histopathology of a skin biopsy with acantholysis, and the relapsing and remitting course. Family history was positive for father and brother. Since she had not responded well in the past to systemic retinoids and did not tolerate the adverse effects of these drugs, we suggested an ablative erbium-YAG laser treatment in general anaesthesia. Laser treatment was performed with the MCL 29 Dermablate (Asclepion Laser Technologies, Jena, Germany) on two occasions. We used a 5 mm focus, pulse energy of 1200 mJ at 8 Hz. The resulting superficial wounds were treated with an ointment containing fusidic acid 0.2% and betamethasone 0.1%. Wound healing was completed after 12 days. No adverse events were observed. CONCLUSIONS: Ablative erbium-YAG therapy is an option for pemphigus chronicus familiaris, in particular in young women and patients who do not tolerate the adverse effects of retinoid therapy.
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spelling pubmed-69107802019-12-17 Successful Treatment of a Widespread Pemphigus Chronicus Familiaris (Hailey-Hailey) By Erbium-YAG-Laser Wollina, Uwe Hansel, Gesina Lotti, Torello Vojvodic, Aleksandra Open Access Maced J Med Sci Case Report BACKGROUND: Familial chronic pemphigus or Hailey-Hailey disease (OMIM 169600) is a rare, autosomal dominant blistering skin disorder the genetic background are mutations of the ATP2 C1 gene. The treatment is challenging. CASE REPORT: A 48-year-old Caucasian female patient presented to the department with a relapse of her Pemphigus chronicus familiaris (Hailey-Hailey). No other medical diseases were known. On examination, we observed an otherwise healthy woman with widespread erosive lesions on the neck, axillae, groins, submammary fold and anal fold. She reported burning sensations and an unpleasant odour. The diagnosis had been confirmed earlier by histopathology of a skin biopsy with acantholysis, and the relapsing and remitting course. Family history was positive for father and brother. Since she had not responded well in the past to systemic retinoids and did not tolerate the adverse effects of these drugs, we suggested an ablative erbium-YAG laser treatment in general anaesthesia. Laser treatment was performed with the MCL 29 Dermablate (Asclepion Laser Technologies, Jena, Germany) on two occasions. We used a 5 mm focus, pulse energy of 1200 mJ at 8 Hz. The resulting superficial wounds were treated with an ointment containing fusidic acid 0.2% and betamethasone 0.1%. Wound healing was completed after 12 days. No adverse events were observed. CONCLUSIONS: Ablative erbium-YAG therapy is an option for pemphigus chronicus familiaris, in particular in young women and patients who do not tolerate the adverse effects of retinoid therapy. Republic of Macedonia 2019-08-30 /pmc/articles/PMC6910780/ /pubmed/31850125 http://dx.doi.org/10.3889/oamjms.2019.764 Text en Copyright: © 2019 Uwe Wollina, Gesina Hansel, Torello Lotti, Aleksandra Vojvodic. http://creativecommons.org/licenses/CC BY-NC/4.0 This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 International License (CC BY-NC 4.0)
spellingShingle Case Report
Wollina, Uwe
Hansel, Gesina
Lotti, Torello
Vojvodic, Aleksandra
Successful Treatment of a Widespread Pemphigus Chronicus Familiaris (Hailey-Hailey) By Erbium-YAG-Laser
title Successful Treatment of a Widespread Pemphigus Chronicus Familiaris (Hailey-Hailey) By Erbium-YAG-Laser
title_full Successful Treatment of a Widespread Pemphigus Chronicus Familiaris (Hailey-Hailey) By Erbium-YAG-Laser
title_fullStr Successful Treatment of a Widespread Pemphigus Chronicus Familiaris (Hailey-Hailey) By Erbium-YAG-Laser
title_full_unstemmed Successful Treatment of a Widespread Pemphigus Chronicus Familiaris (Hailey-Hailey) By Erbium-YAG-Laser
title_short Successful Treatment of a Widespread Pemphigus Chronicus Familiaris (Hailey-Hailey) By Erbium-YAG-Laser
title_sort successful treatment of a widespread pemphigus chronicus familiaris (hailey-hailey) by erbium-yag-laser
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6910780/
https://www.ncbi.nlm.nih.gov/pubmed/31850125
http://dx.doi.org/10.3889/oamjms.2019.764
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