title: Persistent improvement of previously recalcitrant Hailey-Hailey disease with electron beam radiotherapy.
abstract: Hailey-Hailey disease, or familial benign chronic pemphigus, is an autosomal dominant genodermatosis. Disease symptoms may contribute to an adverse impact on quality of life and functional limitation and disability. As Hailey-Hailey disease is chronic and frequently recalcitrant to treatment, multiple therapeutic approaches, including surgical options, have been attempted. We describe here three cases of recalcitrant Hailey-Hailey disease that showed long-term improvement with radiotherapy. Axillary lesions were treated with electron beam at an anti-inflammatory dose (energy 6-8 MeV). Patients received 20 Gy in 10 fractions to 90% isodose, at each axilla. No disease recurrence was observed during a 38 months follow-up of the treated sites. The effect of radiotherapy was thus considered to be locally beneficial, but without any positive influence on the general course of the disease.
authors.affiliation: Department of Dermatology, Medical University of Lodz, Poland. joanna.narbutt@umed.lodz.pl
authors.forename: Joanna
authors.initials: J
authors.lastname: Narbutt
authors.forename: Anna
authors.initials: A
authors.lastname: Chrusciel
authors.forename: Anna
authors.initials: A
authors.lastname: Rychter
authors.forename: Jacek
authors.initials: J
authors.lastname: Fijuth
authors.forename: Aleksandra
authors.initials: A
authors.lastname: Lesiak
authors.forename: Anna
authors.initials: A
authors.lastname: Sysa-Jedrzejowska
mesh_terms: D001706:Biopsy; D019583:Dose Fractionation, Radiation; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D016506:Pemphigus, Benign Familial / Q000473:pathology / Q000532:radiotherapy*; D012008:Recurrence; D012867:Skin / Q000473:pathology / Q000528:radiation effects*; D013997:Time Factors; D016896:Treatment Outcome