Hailey-Hailey disease is an inherited blistering skin condition that can develop at any age, but usually occurs for the first time in people between the ages of 20 and 40. Hailey-Hailey disease usually starts with red spots with small superficial wounds, which mainly occur in the body folds, such as the armpits, groin, buttocks and under the breasts. In the initial phase of the condition, local treatment with a corticosteroid cream may be sufficient.
- What is Hailey-Hailey disease?
- Cause
- Symptoms of Hailey-Hailey disease
- Spots of skin abnormalities
- Blisters and placards
- Examination and diagnosis
- Differential diagnosis
- Skin biopsy
- Hailey-Hailey disease treatment
- Ambient temperature
- Local treatment
- Systemic treatment
- Prevent sweating
- Laser treatment
- Prognosis
What is Hailey-Hailey disease?
Hailey-Hailey disease is a rare hereditary blistering skin disease. The Hailey brothers first described the disease in 1939. It is also called ‘familial benign chronic pemphigus’. This name indicates that this is a basically benign (= benign) condition that persists for a long time (chronic). This skin condition usually runs in families; there are often other people in the family with the same complaints. But the disease can also occur spontaneously. The disease usually manifests itself between the ages of 20 and 40, but it can occur at any age.
Cause
Hailey-Hailey disease is caused by a defect or error in a certain piece of hereditary material, namely the ATP2C1 gene. This gene codes for a special protein that is important for calcium balance in the cell. As a result of this genetic disorder, the skin cells are less able to adhere to each other, causing the skin to peel more easily and cause blisters, wounds and fissures. The skin disease is hereditarily transmitted. Hailey-Hailey disease is an autosomal dominant disease, which means that if one of your parents has the condition, there is a 50% chance that you will develop the condition.
Symptoms of Hailey-Hailey disease
What are the characteristics of Hailey-Hailey disease?
Spots of skin abnormalities
The skin abnormalities often occur in places with a lot of friction and/or (excessive) sweating, such as:
- the neck;
- the armpits;
- under the breasts;
- the elbow creases;
- the groin
- the pubic area; and
- around the anus, the buttock fold.
Blisters and placards
It usually starts with small blisters, blisters or pus heads that quickly dry or break. Subsequently, crusty and wet spots develop. If the condition continues, the spots may thicken (plaques form) and the skin may soften, causing painful cracks to develop. This moist area can be a good breeding ground for bacterial infections. Secondary bacterial infections can lead to an unpleasant odor. The nails may show white stripes lengthwise. Heat, sweating and friction often aggravate the disease, and most patients are more affected during the summer months. In some patients one place is affected and in others the abnormalities are more extensive. Periods of improvement are often alternated with periods of flare-ups of the disease.
Examination and diagnosis
Differential diagnosis
It is sometimes not easy to distinguish Hailey-Hailey disease from other skin diseases that manifest in the skin folds and are associated with inflammation, unless the disease runs in the family and the patient is therefore already familiar with it. Hailey-Hailey disease is often initially mistaken for one of the following conditions:
Atopic eczema in a child with scratch effects / Source: Eisfelder, Wikimedia Commons (CC BY-SA-3.0)
- stains;
- candida; or
- atopic eczema.
Skin biopsy
A biopsy, in which a piece of tissue is removed from the body for tissue examination, is often necessary to make the final diagnosis.
Hailey-Hailey disease treatment
Hailey-Hailey disease is a genetic condition that cannot (yet) be cured. The treatment of this disease is aimed at causing as few complaints as possible.
Ambient temperature
First of all, it is important to wear loose-fitting clothing to reduce friction. It is also important to keep the ambient temperature cool and excessive perspiration can be prevented by wearing cotton clothing.
Local treatment
In the initial phase, local treatment with a corticosteroid cream may be sufficient. Because a secondary bacterial infection regularly occurs in the affected skin area, the use of a topically applied antibiotic may be necessary, possibly combined with an antifungal cream.
Systemic treatment
If local treatment does not provide sufficient relief, systemic medicine can be chosen, for example acitretin, isotretinoin or cyclosporine.
Prevent sweating
Excessive sweating can worsen the symptoms. A good anti-sweat treatment, possibly with botulinum toxin (Botox), can sometimes reduce the complaints. MiraDry is a new and permanent treatment for armpit sweating. This equipment destroys the sweat glands according to the microwave principle.
Laser treatment
Treatment with CO2 laser is also possible. This removes the epidermis from the affected area. A whole new epidermis is formed. Sometimes good and long-lasting results are achieved.
Prognosis
There are no treatments that can make Hailey-Hailey disease go away because it is a genetic condition. Treating the symptoms gives patients relief and reduces infections. The most important part of the treatment is to avoid friction and sweating by wearing light and airy clothing.
read more
- Skin disorders AZ: symptoms, cause and treatment
- Groin spots: red spots, dots or bumps in the groin area
- Armpit: brown or red spots, bump, bumps, itching & rash
- Itching and/or pain under the armpit: with rash, spots and spots
- Red spots on legs and feet, without flaking
