Treatment of Dowling Maera Type of Epidermolysis Bullosa Simplex by Oral Erythromycin

Dowling Meara type of epidermolysis bullosa simplex (EBS-DM) is a rare genodermatosis due to keratin 5 and 14 mutation, characterized by skin fragility and spontaneous or post traumatic blisters. Neonatal period and infancy are critical since this autonomic dominant affection usually improves with age. Cyclins seem to be efficient in some cases of EBS but are prohibited in children younger than 8 years old. Erythromycin can be a good alternative in this population due to its antibacterial and anti-inflammatory potential. The aim of this study is the evaluation of the efficiency of oral erythromycin to decrease the number of cutaneous blisters in severe EBS-DM patients from 6 months to 8 years old after 3 months of treatment. Primary end point is the number of patients with decrease of blisters' number of at least 20% after 3 months of treatment by oral erythromycin. It is a preliminary study on 8 patients. Treatment is oral erythromycin twice a day during 3 months. Follow up for each patient is 5 months. The duration of the study is 1 year.

number of patients with decrease of blisters' number of at least 20% after 3 months of treatment by oral erythromycin

Principal end point is evaluated at inclusion and after one month of treatment, 3 months of treatment and 2 months after the end of the treatment

Secondary end points are : effect of 3 months of oral erythromycin on - Global tolerance of treatment.

For each patient and globally, the nature, the frequency and the severity of the various unwanted effects will be described on the duration of the study.

These criteria will be analyzed in comparison with the values to the inclusion (M0). We shall try to estimate the obstinacy of an effect 2 months after the end of the treatment.

These criteria will be analyzed in comparison with the values to the inclusion (M0). We shall try to estimate the obstinacy of an effect 2 months after the end of the treatment.

Inclusion Criteria: Severe Dowling Meara EBS patients (2 or more new blisters a day) signature of informed consent Patient of 2 sexes Age from 6 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 lit(enlightened) by the relatives(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 Exclusion Criteria: Patient allergic to the erythromycin Patient presenting an intolerance to the fructose, a syndrome of malabsorption some glucose and some galactose or a deficit sucrase-isomaltase Renal and\or hepatic Insufficiency Patient taking a medicine against indicated or misadvised in association with the erythromycin