Dowling Maera's bullous epidermolysis is a génodermatose with autosomique dominant transmission owed to transfers of the genes coding for keratins. It results from it a cutaneous fragility very severe especially during the early childhood. Tetracyclines showed a certain efficiency in cases isolated probably by their anti-inflammatory action but cannot be used at the young child's. The érythromycine, used in the other inflammatory dermatosis, seems to be a good candidate for these patients. MedD
Conditions
Interventions
Trade Name: erythrocine
Product Name: erythrocine oral
Pharmaceutical Form: Granules for syrup
Trade Name: erythromycine
Product Name: erythrocine oral
Pharmaceutical Form: Granules for syrup
Sponsors
CHU de NICE
Eligibility
Sex/Gender
All
Inclusion criteria
Inclusion criteria: - Patient of 2 sexes - Carrier of a severe EBS-DM (more than 2 new bubbles a day on average) - Age from 3 months to 8 years. From this age we consider that the patient will less need this treatment or can take cyclines. - Systematic Obtaining of the consent by the parents of the child, after information about the objectives and the constraints of the study. - Agreement of the minor - Patient member to the Social Security Are the trial subjects under 18? yes Number of subjects for this age range: F.1.2 Adults (18-64 years) no F.1.2.1 Number of subjects for this age range F.1.3 Elderly (>=65 years) no F.1.3.1 Number of subjects for this age range
Exclusion criteria
Exclusion criteria: Patient allergic to the érythromycine - Patient presenting an intolerance to the fructose, a syndrome of bad absorption some glucose and some galactose or a deficit it sucrase-isomaltase - Renal and\or hepatic insufficiency - Patient taking a medicine against indicated or disadvised in association with the erythromycin -
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Main Objective: The main objective is to estimate the efficiency of the oral érythromycine to decrease the number of cutaneous bubbles at the patients affected by EBS-DM after 3 months of treatment.;Secondary Objective: The secondary objectives are: - To estimate the efficiency of the oral érythromycine in 3 months for: - Decrease the affected surface, - Decrease the prurit, decrease the cutaneous fragility - To estimate the global tolerance of the treatment. - To estimate the efficiency in term of improvement of the symptomatologie bulleuse, the affected surface and the prurit 2 months after the stop of the treatment, at M5, to estimate the remote preservation of a possible efficiency of the oral érythromycine. - To look for a bacterial colonization by bacteriological takings to M0, M1, M3 and M5, with study of the antibiogramme to detect a possible resistance in the érythromycine as well as its remote preservation. ;Primary end point(s): The main criterion will be patient's rate presenting a decrease of the average number of new cutaneous bubbles a day of at least 20 % after the period of treatment. The number of new bubbles will be counted during a week by the parents on a patient nude, on all the cutaneous surface of the body (including the scalp) before the daily care of drilling of bubbles and bandage. He will be retranscribed in the pad put back(handed) to the patient or to parents. Any new bubble must be counted whatever is its size. A bubble will thus be counted only once, the day of its appearance. The average of the number of new bubbles a day will then be calculated by the investigator. | — |
Countries
France
Outcome results
None listed