Injections of Botulinic Toxin in Plantar Lesions of Localized Epidermolysis Bullosa Simplex
Injections of Botulinic Toxin in Plantar Lesions of Localized Epidermolysis Bullosa Simplex
The investigators hypothesize that palmar injections of botulinic toxin, via an inhibition of
 the sudation, would limit the occurrence of blisters in localized epidermolysis bullosa
 simplex (LEBS).
Epidermolysis bullosa is a group of rare genetic diseases characterized by the occurrence of
 blisters and erosions due to skin fragility. There are 4 different subgroups, based on the
 location of the skin cleavage area. The most frequent subgroup is the simplex form,
 consisting predominantly of the localized form (localized epidermolysis bullosa simplex:
 LEBS). The incidence of LEBS was estimated at between 1/318.000 and 1/35.000. The disease
 starts early in infancy by the occurrence of blisters and erosions located on soles,
 secondary to frictions during the walk. The phenomenon is worsened by heat and sudation. LEBS
 is due to mutations in keratin genes. Life expectancy in LEBS is normal but the quality of
 life is significantly impaired due to permanent skin pain and limitation of everyday
 activities (walking, sports). There is no effective or curative treatment. Patients must
 limit the frictions, protect the skin and use plasters in case of skin lesions.

 Botulinic toxin has an agreement for the treatment of axillary hyperhidrosis and has been
 shown to be also effective on palms and soles. The efficacy of botulinic toxin in plantar
 lesions of LEBS has been reported in the literature (one case report and a short
 retrospective series of 6 patients) but there is no proper study.
Inclusion Criteria:

 -  Diagnosis of LEBS based on clinical symptoms and in some cases histological or
 molecular findings

 -  Palmar skin lesions: blisters and/or : erosions, edematous and erythematous lesions,
 crusts. 3 lesions per foot, as a minimum

 -  Similar clinical severity of skin lesions on both feet

 -  Patient with social security

 -  Written consent of the patient

 -  Patient able to understand the study's questionnaires

 Exclusion Criteria:

 -  Patients with only one leg and a different number of toes on each foot.

 -  Known hypersensitivity to botulinic toxin or its excipients

 -  Current treatment with aminosides

 -  Myasthenia

 -  Swallowing difficulties

 -  Respiratory disorders

 -  Past medical history of dysphagia or pneumopathy of inhalation

 -  Known allergy or contraindications to lidocaine, prilocaine, paracetamol or nitrous
 oxide

 -  Pregnancy (positive pregnancy test (β-HCG) for women of childbearing age, performed
 within the 2 days prior to the study. Breastfeeding.

 -  Contraception during 6 months from inclusion

 -  Mental or physical or judicial incapacity to fill the questionnaires

 -  Guardianship patients

 -  Skin infection on the soles at the time of the inclusion

 -  Skin lesions located on the soles, not related to LEBS (ie. post traumatic wound,
 wart)

 -  Patient suffering from dishydrosis

 -  Botulinic toxin injections in the previous 6 months

 -  Inclusion in another study in the previous 2 months