Papular acantholytic dermatosis (PAD), is a very rare, benign, and commonly asymptomatic dermatosis of the anogenital region. PAD presents with multiple discrete whitish, pink, or skin-colored papules that may coalesce to form plaques. They are usually asymptomatic, but pruritus or a burning sensation may be associated. Sites of predilection include the genital skin, perineum, perianal skin, and the inguinal folds. Lesions persist for months or years.

PAD is slightly more commonly reported in women than in men. It typically has onset in the third to seventh decade of life, although pediatric cases may rarely occur. PAD occurs sporadically.

The etiology and pathogenesis of PAD is still unknown. Some have suggested that PAD represents a localized form of Hailey-Hailey disease or Darier disease. Genetic mutations such as ATP2C1 and ATP2A2 have been reported in 3 cases, and in another case, PAD developed in a patient with a family history of Hailey-Hailey disease. Further investigation is needed to verify this hypothesis.