Apremilast in combination with botulinum toxin-A injection for recalcitrant Hailey-Hailey disease

Abstract

Hailey–Hailey disease (HHD) is a rare genodermatosis caused by a mutation in the ATP2C1 gene, which codes for a calcium channel. This ionic alteration leads to a defective keratinocyte adhesion. Hailey–Hailey disease is characterized by recurrent flare-ups of vesicles and flaccid blisters in folds that have an impact on patients’ quality of life. The recurrent and refractory nature of this condition makes its treatment a challenge. Isolated cases have been reported describing controversial results after treatment with apremilast. Here we present a case of refractory HHD who experienced an excellent response after treatment with apremilast and botulinum toxin-A (BoNT) infiltrations.