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.
Study Type
INTERVENTIONAL
Allocation
NA
Purpose
OTHER
Masking
NONE
Enrollment
30
Injection of 50 UI of botulism toxin for treated zone
Evaluation of quality of life measured by change in the DLQI score
Variation of DLQI score between Baseline and M1
Time frame: Day 0 and day 30
Evaluation of quality of life measured by change in the DLQI score
Variation of DLQI score between Baseline and M3
Time frame: Day 0 and day 90
Evaluation of quality of life measured by change in the DLQI score
Variation of DLQI score between Baseline and M6
Time frame: Day 0 and day 180
Evaluation of skin improvement in treated areas using change the IGA score
Variation of IGA score between Baseline and M1
Time frame: Day 0 and Day 30
Evaluation of skin improvement in treated areas using change the IGA score
Variation of IGA score between Baseline and M3
Time frame: Day 0 and Day 90
Evaluation of skin improvement in treated areas using change the IGA score
Variation of IGA score between Baseline and M6
Time frame: Day 0 and Day 180
Evaluation of psychosocial impairment at measured by change in the HidroQoL score
Variation of HidroQoL score between Baseline and M1
Time frame: Day 0 and Day 30
Evaluation of psychosocial impairment measured by change in the HidroQoL score
Variation of HidroQoL score between Baseline and M3
Time frame: Day 0 and Day 90
Evaluation of psychosocial impairment measured by change in the HidroQoL score
Variation of HidroQoL score between Baseline and M6
Time frame: Day 0 and Day 180
Evaluation by the investigator of the treated lesions global severity change as assessed by comparison using measurement of the affected area
Variation of treated lesions severity between Baseline and M1
Time frame: Day 0 and Day 30
Evaluation by the investigator of the treated lesions global severity change as assessed by comparison using measurement of the affected area
Variation of treated lesions severity between Baseline and M3
Time frame: Day 0 and Day 90
Evaluation by the investigator of the treated lesions global severity change as assessed by comparison using measurement of the affected area
Variation of treated lesions severity between Baseline and M6
Time frame: Day 0 and Day 180
Evaluation of patient's satisfaction Using the IGA score " Improvement Global Assessment "
Time frame: Day 180
Evaluation of patient treatment acceptability using visual analogic pain scale
Time frame: Day 0 after injection
Evaluation of acceptability over the medium to long term as assessed by occurence of side effects
Time frame: Day 30
Evaluation of acceptability over the medium to long term as assessed by occurence of side effects
Time frame: Day 90
Evaluation of acceptability over the medium to long term as assessed by occurence of side effects
Time frame: Day 180
Evaluation of long term efficacy as assessed by percentage of non-responder patients with IGA score egal to 0
Time frame: Day 30
Evaluation of long term efficacy as assessed by delay for significant relapse (reappearance of skin lesions justifying treatment)
Time frame: Up to 180 days
Evaluation of long term efficacy as assessed by comparison between the number of infection episodes occurred during the 6 months before the study or during the 6 months of the study
Time frame: Up to 180 days
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