Multiple seborrheic keratosis in a patient with familial benign pemphigus

Hailey-Hailey disease (HHD) or familial benign chronic pemphigus is a dermatosis with an autosomal dominant inheritance characterized by full penetrance and variable expressivity: from individual resembling eczema lesions to extensive lesions of the skin. HHD causes mutations in the gene ATP2C1, which encodes the ATPase calcium-magnesium secretory mechanism of a human [1,2]. The mechanism by which mutated ATP2C1 causes acantholysis, still not installed. Clinically, the disease manifests often at the age of 30-40 years in the form of painful erosions and scaly erythematous plaques in the intertriginous areas. Subjectively severe itching concerns, resulting in scratching and painful cracking. Conjunctiva, mucosa, and vulva involvement is rarely seen. Defeat folds accompanied by an unpleasant odor. After healing it is characterized by hyperpigmentation. The acute illness usually occurs under the influence of increased sun exposure, sweating, friction, irritating therapy. Remission can be from several months to several years. Histologically, HHD is similar to Darier disease: find suprabasal separation of the epidermis caused by acantholysis and resulting in lacunae or bullae, and upward proliferation of papillae as so-called villi into the lacunae.