Evaluation of the Improvement of Quality of Life of Patients Suffering From Hailey Hailey or Darier Disease After Injections of Botulism Toxin Into Large Folds.
Completed
University Hospital, Toulouse
Phase 1
2015-09-01
Hailey Hailey and Darier disease are rare genetic dermatoses. Mutations of 2 genes (ATP2C1 or
ATP2A2 respectively) are responsible for the diseases. These genes have a key role in calcium
pump; their defect create abnormal link between keratinocytes' desmosomes and induce skin
lesions. Clinically, patients present with inflammatory lesions located in the folds. Quality
of life is impaired because of pain, pruritus and tendency to infections. Lesions are
permanent but acute exacerbations occur in hot seasons because of increased sweating. Usual
therapies are often not effective (local treatment, laser, phototherapy). Because sweating is
a well established inducing or aggravating factor, botulism toxin could be an effective
treatment for these diseases.
Botulism toxin is already used in clinical practice and acts via a decreased sweet secretion.
Improvement of skin lesions in Hailey-Hailey or Darier diseases has been previously reported
in a few cases but there is no study properly evaluating the benefit of such treatment.
The aim of the project is to study the improvement of quality of life for patients suffering
from Hailey-Hailey or Darier diseases after a injections of botulism toxin in large skin
folds. The principal objective is to estimate the distribution of the variation of quality of
life at M1 vs. baseline.
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