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Tytuł pozycji:

Botulinum toxin injections as an effective treatment for patients with intertriginous Hailey-Hailey or Darier disease: an open-label 6-month pilot interventional study

Tytuł :
Botulinum toxin injections as an effective treatment for patients with intertriginous Hailey-Hailey or Darier disease: an open-label 6-month pilot interventional study
Autorzy :
Isabelle Dreyfus
Aude Maza
Lauriane Rodriguez
Margot Merlos
Hélène Texier
Vanessa Rousseau
Agnès Sommet
Juliette Mazereeuw-Hautier
Pokaż więcej
Temat :
Botulinum toxin
Hailey hailey disease
Darier disease
Quality of life
Medicine
Źródło :
Orphanet Journal of Rare Diseases, Vol 16, Iss 1, Pp 1-9 (2021)
Wydawca :
BMC, 2021.
Rok publikacji :
2021
Kolekcja :
LCC:Medicine
Typ dokumentu :
article
Opis pliku :
electronic resource
Język :
English
ISSN :
1750-1172
Relacje :
https://doaj.org/toc/1750-1172
DOI :
10.1186/s13023-021-01710-x
Dostęp URL :
https://doaj.org/article/05374b0a600e41e7bd982e8fd8718dc8
Numer akcesji :
edsdoj.05374b0a600e41e7bd982e8fd8718dc8
Czasopismo naukowe
Abstract Background Patients with Hailey-Hailey and Darier diseases present with disabling inflammatory lesions located in large skin folds, which are often exacerbated or induced by sweating. Quality of life is highly impaired because of pain and recurrent skin infections. An improvement in skin lesions after botulinum toxin A injections has previously been reported in some patients but no prospective interventional studies are available. The aim of this open-label, 6-month, interventional pilot study (NCT02782702) was to evaluate the effectiveness and safety of botulinum toxin A for patients with moderate to very severe skin lesions located in folds. Results Thirty patients (26 Hailey-Hailey/4 Darier) were included. Botulinum toxin A proved effective within the first month in two-thirds of patients, taking all study parameters (itchiness, cutaneous pain, sweating and odour, infections, psychosocial impairment and quality of life) into account and persisted during the 6-month follow-up period. No patient was classed as a BtxA non-responder, but 11 (37%) Hailey-Hailey patients (the most severe ones), experienced a relapse during the study. No serious side effects were reported. Mild transient clear fluid discharge at the site of the injections was reported for 27% of patients. Conclusions Botulinic toxin seems to be an effective and safe treatment for Hailey-Hailey and Darier diseases. Nevertheless, it may prove insufficient for the severest of Hailey-Hailey cases and could be considered as supplementary to other conventional treatments. Further studies are required to confirm our results on larger Darier cohorts.
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