Treatment of Hailey-Hailey disease with oral erythromycin

Haliey-Hailey disease is a rare autosomal dominant acantholytic disorder characterized from late adolescence or adulthood by recurrent eruptions of vesicles and blisters usually located on the neck, axillae and groin. The clinical cases of four unrelated adult male patients with Hailey-Hailey disease are presented. In all patients, a relatively short-course treatment with oral erythromycin (3-4 weeks) induced a long-lasting remission (8 months). Since bacteriological investigations excluded local infection, other hypothetical pharmacological effects (anti-inflammatory action and inhibition of cytokine release), besides the antibacterial properties of erythromycin, should be considered to explain such clinical improvement. However, until precise information about the pathogenesis of Hailey-Hailey disease is available, the mechanism of action of erythromycin in this disease remains speculative. Further studies in larger series of patients are needed in order to assess the role of erythromycin, and perhaps other macrolides, as a standard therapeutic option for Hailey-Hailey disease.