Hailey-Hailey Disease (Familial Benign Chronic Pemphigus
[Autosomal Dominant]): A family history usually is present. The onset of age is usually
in the fourth and fifth decade. It is characterized by the presence of vesicles and
erythemateous plaques with overlying crusts typically in the genital areas, also seen in
the chest, neck and axillary regions. Burning and itching most often accompany the
eruption, and a malodorous drainage occurs as a result of secondary infection. Symptoms
related to staphylococcal and candidal overgrowth are common. The characteristic clinical
appearance, as well as biopsy, readily confirms the diagnosis. It has been suggested that
Hailey-Hailey
disease results from a genetic defect in a calcium pump protein. The
gene is localized on chromosome 3. The dotted pattern is lost in
acantholysed and perilesional areas and antidesmoplakin I + II-positive proteins are
observed diffusely in the cytoplasm.