Familial benign chronic pemphigus (Hailey-Hailey disease) treated with electron beam radiation

EBRT: electron beam radiation therapy GRT: Grenz ray therapy HHD: Hailey-Hailey disease INTRODUCTION Hailey-Hailey disease (HHD), or familial benign chronic pemphigus, is an autosomal dominant genodermatosis. In this condition, dysregulation of intracellular calcium homeostasis leads to impaired desmosome function and suprabasilar acantholysis of the epidermis. Clinically, this condition presents as intertriginous vesicles, erosions, and weeping, fissured plaques. The painful nature of HHD, risk of secondary infection, and malodor can significantly affect a patient’s quality of life. The lesions are often difficult to control with topical steroids and antimicrobials, necessitating more aggressive treatments. In previous case reports, ionizing radiation was found to prolong remission of plaques. We report the use of electron beam radiation therapy (EBRT) to treat 2 patients with recalcitrant HHD.