Content » Vol 90, Issue 2

Clinical Report

Persistent Improvement of Previously Recalcitrant Hailey-Hailey Disease with Electron Beam Radiotherapy

Joanna Narbutt, Anna Chrusciel, Anna Rychter, Jacek Fijuth, Anna Sysa-Jedrzejowska, Aleksandra Lesiak
DOI: 10.2340/00015555-0791

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.

Significance

Supplementary content

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