Atypical Manifestation Of Hailey-hailey Disease

Hailey-Hailey disease(familial benign chronic pemphigus) is a rare intraepidermal blistering disorder, which is inherited as an autosomal dominant trait. It is clinically characterized by recurrent vesicles which rupture, leaving painful fissures(rhagades) and scaly erythematous plaques on the neck, axilla, groin, and in intertriginous areas. In recent years, the atypical cases can be observed in the clinic gradually, including rare and atypical lesions such as maculopapule, keratosis papules, papillomatosis, discrete punctate palmar keratoses. The aims of this paper were to offers an update on the knowledge about the age of onset,genemutation,disease modifying factors,pattern of disease and clinical manifestations, histopathoiogy, complications and treatment of Hailey-Hailey disease.