Hailey–Hailey disease

The Hailey - Hailey is a skin disease in which there is a akantholytischen blistering due to a congenital ( inherited ) disorder of keratinization of the skin.

This means that the cells that control the callus formation and in the prickle cell layer ( seed layer ) are the epidermis (outer skin), peel off this layer, so that in this way bubbles. This is usually in the armpits and groin.

Were first described in 1939, the American dermatologist and brothers William Howard Hailey ( 1898-1967 ) and Hugh Edward Hailey ( 1909-1964 ).

Synonyms

Chronic recurrent acantholysis, dyskeratosis bullosa hereditaria, familial benign pemphigus, Hailey - Hailey disease, Hailey - Hailey 's disease, Gougerot - Hailey - Hailey disease, pemphigus benign familial, recurrent dermatitis herpetiform

Causes

Hailey - Hailey is autosomal dominant. A group of RWTH Aachen dismissed in 2003 by a mutation of the gene ATP2C1.

Heat, profuse sweating and microbial infections may contribute to the development of this skin disease, as well as exposure to UV radiation, ie, a UV irradiation of the skin, such as occurs while sunbathing.

Symptoms

Symptomatic of Hailey - Hailey is the formation of erythema (skin redness ) that occur due to an increased blood flow in the context of inflammatory processes, and bubbles. Both occur in the armpits, neck, groin and anal area. In addition, it comes to pigment changes and skin discoloration. The skin symptoms are often accompanied by severe itching and tendency to superinfection, meaning that the risk of recurrent bacterial infections is to the inflamed sites.

Differential diagnoses

Diseases that can be easily confused with Hailey Hailey and their presence must therefore be excluded for diagnosis, are erythrasma, Candidaintertrigo, an excessive moisture, such as profuse sweating, favored fungal infection of the skin, as well as pemphigus vegetans, an equally blistering associated, usually chronic, severe skin disease still unknown reasons and anal eczema.

Therapy

The prevention of skin-to -skin contact is reduced, the mechanical irritation to the skin by rubbing in movement. At medicinal antiseptic preparations, ie agents which are used on the skin to infection, antibiotics and corticosteroids are used. Other measures, such as dermabrasion called removing the top layers of skin affected by laser or plastic surgery can cause long-term improvement.