Generalized familial benign chronic pemphigus (Hailey-Hailey disease) treated successfully with low-dose naltrexone

Familial benign chronic pemphigus or Hailey-Hailey disease (HHD) is an autosomal dominant genodermatosis with complete penetrance that was initially described by dermatologists and brothers Howard and Hugh Hailey.1 This disorder results from mutations in the ATP2C1 gene on chromosome 3q21, which encodes the Golgi-associated Ca2+ ATPase. Mutations in this gene lead to abnormal intracellular Ca2+ signaling leading to impaired processing of junctional proteins needed for cell-cell adhesion.