Treatment of Hailey-Hailey disease with oral erythromycin

Hailey-Hailey disease is a rare autosomal dominant acantholytic disorder ch aracterized from late adolescence or adulthood by recurrent eruptions of ve sicles and blisters usually located on the neck, axillae and groin. The cli nical cases of four unrelated adult male patients with Hailey-Hailey diseas e are presented. In all patients, a relatively short-course treatment with Oral erythromycin (3-4 weeks) induced a longlasting remission (8 months). S ince bacteriological investigations excluded local infection, other hypothe tical pharmacological effects (anti-inflammatory action and inhibition of c ytokine release), besides the antibacterial properties of erythromycin, sho uld be considered to explain such clinical improvement. However, until prec ise information about the pathogenesis of Hailey-Hailey disease is availabl e, the mechanism of action of erythromycin in this disease remains speculat ive. Further studies in larger series of patients are needed in order to as sess the role of erythromycin, and perhaps other macrolides, as a standard therapeutic option for Hailey-Hailey disease.