Treatment of Epidermolysis Bullosa Dystrophica by Polyphenon E (Epigallocatechin 3 Gallate)

Study Overview
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Brief Summary
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Dystrophic epidermolysis bullosa hereditaria are genodermatosis responsible for formation of cutaneous bullous lesion arising spontaneously or after mechanical trauma. These lesions are due to mutation on gene COL7A1 coding for collagen VII. There is no treatment available. Cares are consisting to dress lesions and to protect the skin. The investigators have recently observed on patients having residual expression of collagen VII that phenotype severity is modulated by activation degree of dermic metalloproteinase. The investigators have also observed that epigallocatechin-3-gallate (Polyphenon E®) could be regulated this activity. The primary purpose of this study is to assessing the efficacity of Polyphenon E to decrease the number of cutaneous bullosa after four month of treatment. The primary outcome measure is the rate of patient presenting a decrease of 20% or more of the number of cutaneous bullosa. Secondary outcomes are: severity of mucosa impairment, affected cutaneous surface, the average duration of cicatrisation and treatment tolerance. This study foresees the inclusion of 22 patients older than 2 years old in 5 centers. When patients are included, they will be randomized and receive the treatment (or placebo) for 4 months.

Official Title
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Treatment of Epidermolysis Bullosa Dystrophica by Polyphenon E (Epigallocatechin 3 Gallate)

Conditions
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Epidermolysis Bullosa Dystrophica

Intervention / Treatment
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* Drug: Polyphenon E before Placebo
* Drug: placebo before treatment


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: known mutation of COL7A1 Exclusion Criteria: tea drinkers patient receiving induction treatment,protease inhibitor treatment liver failure

Ages Eligible for Study
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Minimum Age: 2 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Treatment
Allocation: Randomized
Intervention Model: Crossover Assignment
Masking: Double


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Other: 1<br>patients receive the treatment in first and placebo in second part of study | Drug: Polyphenon E before Placebo<br>* patients receive polyphenon E during 4 months, then 2 months of wash-out and finally 4 months of placebo<br>|
| Other: 2<br>patients receive placebo in first and treatment in second part of study | Drug: placebo before treatment<br>* patients receive 4 months of placebo, then 2 months of wash out et finally 4 months of treatment<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| decrease of number of cutaneous bullosa |  | after 4 months of treatment | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| efficacity of treatment |  | at 4 , 6, 7 10 months after beginnig of treatment and at year 1 | 
| tolerance tio treatment |  | at 1, 4, 6, 7, 10 and 12 months after beginnig the treatment |