Familial benign chronic pemphigus (Hailey-Hailey disease).

Abstract

Familial benign chronic pemphigus (Hailey-Hailey disease) is transmitted through an autosomal dominant gene that regulates the passage of the calcium through the cell membrane (the calcium pump) and is responsible for the integrity of the epidermis. The disease usually starts in the third to fourth decade of life and the heat is a risk factor; for this reason significantly the disease worsens in summer and predominantly affects the folds.
The differential diagnosis from pemphigus vulgaris is based on familiarity, no mucosal involvement and negative immunofluorescence; Darier disease has a more pronounced dyskeratosis with round bodies and grains. The therapy is based on topical immune suppressive drugs (potent corticosteroids, tacrolimus), in severe cases even systemic (cyclosporine). Good results were reported with various therapies, including pulsed dye laser (1, 2) and isotretinoin.