Background: Hailey-Hailey disease (HHD) is characterised by episodes of weeping erythematous lesions, particularly in areas subject to friction or maceration. Treatment is complex. The value of botulinum toxin has been demonstrated in several studies and in individual cases.
Aim: To report clinical and progressive data for 8 patients treated for HHD with injections of botulinum toxin A (BTX-A), following the failure of several other therapeutic approaches.
Patients and methods: Eight patients (three males and five females), of median age 52.5 years (31-80), were included in this retrospective study. Familial history of the disease was noted in 75% of cases. The lesions affected the axillary regions (62% of cases), the sub-mammary region (almost all female patients), the inguinal region (75%) and the genital area (25%). The mean dose injected per site and per session was 300IU of Dysport®. Clinical evaluation was based on photographs taken before treatment and then after 6 months.
Results: Effects on sweating were rapid and occurred as of the fourth day treatment. On average, patient felt the benefits of the injection within 7 days, with subsidence of their erythema and healing of the rhagades. At 6 months, complete clinical response was noted in 80% of the treated zones (12 sites of 15), with partial response in 3 profuse zones (sub-mammary and inguinal). Maintenance sessions were initiated for 6 of the 8 patients due to relapse beyond six months.
Conclusion: Botulin toxin appears to offer a therapeutic alternative in resistant forms of HHD, either as follow-on treatment or as an adjuvant to more radical forms of therapy such as CO2 laser. These retrospective data, as well as the optimal doses and injection rates, require further refinement by means of prospective studies.
Keywords: Botulinum toxin; CO2 laser; Familial benign pemphigus; Hailey-Hailey disease; Laser CO2; Maladie de Hailey-Hailey; Pemphigus bénin familial; Sudation; Toxine botulique.
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