Hailey-Hailey disease
| Classification according to ICD-10 | |
|---|---|
| Q82.8 | Other specified congenital skin malformations |
| ICD-10 online (WHO version 2019) | |
The Hailey-Hailey disease is a skin disease, which is due to a congenital keratinization (inherited) of the skin to a acantholytic comes bubbling.
This means that the cells that control the formation of the cornea and are located in the prickly cell layer (germ layer) of the epidermis ( epidermis ) detach themselves from this layer, so that blisters are formed. This is usually done in the armpits and groin area.
The US dermatologists and brothers William Howard Hailey (1898-1967) and Hugh Edward Hailey (1909-1964) were the first to describe it in 1939 .
Synonyms
Chronic recurrent acantholysis, dyskeratosis bullosa hereditaria, familial benign pemphigus, Hailey-Hailey disease, Hailey-Hailey syndrome, Gougerot-Hailey-Hailey disease, pemphigus chronicus benignus familiaris, recurrent herpetiform dermatitis
causes
Hailey-Hailey's disease is inherited as an autosomal dominant trait. In 2003, a group from RWTH Aachen University demonstrated a mutation in the ATP2C1 gene.
Heat, profuse sweating and microbacterial infections can promote the development of this skin disease, as can UV exposure, i.e. UV radiation of the skin, as occurs when sunbathing.
Symptoms
Symptomatic of Hailey-Hailey's disease is the formation of erythema (reddening of the skin), which occurs as a result of increased blood flow as part of inflammatory processes, and blisters. Both occur in the armpits, neck, groin, and anal area. In addition, there are pigment changes and discoloration of the skin. The skin symptoms are often accompanied by severe itching and are prone to superinfection , which means that there is a risk of recurring bacterial infections in the inflamed areas.
Differential diagnoses
Diseases that can easily be confused with Hailey-Hailey's disease and whose presence must therefore be ruled out for diagnosis are erythrasma , Candidaintertrigo , a fungal infection of the skin that is favored by excessive moisture, such as profuse sweating, and pemphigus vegetans , also with Blistering associated, mostly chronic, severe skin disease of still unexplained cause and anal eczema .
therapy
Avoiding skin-to-skin contact reduces mechanical irritation of the skin from chafing when moving. Antiseptic preparations, i.e. agents that are used on the skin against infections, antibiotics and cortisone preparations are used as drugs . Further measures such as removing the affected uppermost layers of skin using a laser or plastic surgery, called dermabrasion , can bring about improvement.
