Hailey-Hailey Disease

The Hailey-Hailey disease is also known as familial benign chronic pemphigus. The Hailey brothers originally described it. It is a rare inherited skin condition in which there are red scaly areas that can be itchy and sore, which can lead to superficial blisters and eroded (broken) areas of the skin.

This condition usually affects the folds of the groin, armpits, neck, and under the breasts. The condition flares intermittently and tends to come and go. Many patients can lead full and everyday lives, with their condition being a nuisance rather than a serious problem.

Some people are more severely affected and experience more persistent painful raw areas of the skin with the development of superficial blisters.

The disease is caused by a change in the DNA of a gene on chromosome 3. Usually, this gene helps the cells in the outer layer of the skin (the epidermis) to stick together. In Hailey-Hailey disease, this adhesion of cells is weakened, resulting in separation of the cells in the surface layer of the skin.

This condition happens mostly in areas where the skin is rubbed, such as the groin and under the arms. The altered gene runs in families and affects both men and women equally. 

The Hailey-Hailey disease is not contagious and is not a result of allergies. There is a tendency for Hailey-Hailey disease to get worse with sweating and friction, as well as in hot weather.

Yes. The condition is inherited in a pattern known as ‘autosomal dominant inheritance,’ which means that there is a 1 in 2 [50:50] chance that each child of an affected parent will inherit the skin problem. The severity may vary between affected members of the same family. While it is hereditary, not all patients have affected family members.

During a flare-up, the affected skin may become uncomfortable, with burning and itching symptoms. This problem is aggravated on skin surfaces continually rubbing against each other, such as in the groin while walking.

Flare-ups tend to settle down in a few days with appropriate treatment, although sometimes they may improve without treatment. Severe and painful outbreaks in an area such as the groin, genital area, or in the crease of the buttocks may make walking and work so uncomfortable that an individual may need a few days of rest.

Severe flare-ups may lead to infection and discharge, which may cause increased pain or a strong smell from the affected areas. The affected areas may become particularly problematic if secondarily infected. This condition may be due to bacterial or viral (particularly the cold sore virus) infection.

The condition usually first appears between the ages of 15 and 40 years, but it may begin at any age. The severity of the disease varies widely and is unpredictable. Red, scaly areas, superficial blisters, or raw areas appear at sites of friction, especially at the sides of the neck and in the skin folds (under the arms, groin, or under the breasts). 

The skin looks normal in-between flare-ups and does not scar. However, some patients may find that there is a brown discoloration that can occur as a result of the inflammation (post-inflammatory hyperpigmentation), which can take months to fade. Some people notice that their condition improves as they get older.

It is very common for the condition to be mistaken for other skin conditions such as eczema, psoriasis, contact dermatitis (due to an allergy), fungal infection, or impetigo (a bacterial skin infection). The diagnosis of Hailey-Hailey disease can be confirmed by taking a sample of the skin (a biopsy) for examination under the microscope.

 A second biopsy is often taken for direct immunofluorescence to confirm the diagnosis.

No. The underlying genetic defect cannot be altered; however, treatment does help with the prevention of flare-ups and may increase the speed of healing.

There are several ways of helping patients with Hailey-Hailey disease: 

• Corticosteroid anti-inflammatory creams can be beneficial and may be prescribed in combination with an antibiotic ointment to treat skin infection. A pricking or itching sensation is usually present when a flare-up is starting, and the corticosteroid cream may prevent the rash from developing any further if the cream is applied immediately. Enough of the cream should be applied to cover the affected area. The cream only needs to be used once or twice a day on the affected skin. There is no need to put the steroid cream on unaffected skin.
• Careful washing is essential with an antiseptic soap substitute or antiseptic bath oil to prevent infection occurring.
• Topical antibiotics, e.g., clindamycin, mupirocin, or antifungal, e.g., ketoconazole creams can be helpful for short periods such as a week or two. Long-term antibiotic creams are best avoided to reduce the risks of germs becoming resistant. 

• There is an increased chance of developing a skin infection if you have Hailey-Hailey disease. Infection with bacteria such as Staphylococcus aureus may occur and require treatment with oral antibiotics prescribed by your doctor.
• The “cold sore” virus (herpes simplex) sometimes causes skin infection in patients with Hailey-Hailey disease. If the condition suddenly gets worse and is much more painful than usual, this may be a sign of a cold sore virus infection. You should contact your GP or dermatologist immediately if this happens, as you may need oral anti-viral treatment.
• Sometimes oral (taken by mouth) treatments that affect the immune system may need to be used (e.g., oral corticosteroids, ciclosporin, acitretin, or methotrexate. These drugs have potentially serious side effects and are only prescribed for patients who do not respond to first-line topical treatment or oral antibiotic treatment.
• There is new evidence for the use of magnesium chloride oral solution. Naltrexone, which inhibits opioid pain-relieving receptors, may also be of help. It is not clear yet how this works. Other treatments:
• Botox (botulinum toxin) is an injection that can help alongside topical treatments. It works by reducing sweating and keeping the area dry, and reduce the risk of infection.
• Topical photodynamic therapy, a type of light treatment, has been reported to help in some resistant cases. This treatment involves the application of a cream to the affected skin that sensitizes the skin cells to light, followed by exposure to a special red lamp 3 hours later. Other possible approaches to treatment include the removal of affected areas surgically (which may result in scar formation), dermabrasion, and certain forms of laser treatment.

Heat, sweating, or friction may make the rash develop; it is sensible to try and avoid these where possible. For example, it may be helpful to wash a new shirt to soften a stiff collar before wearing it. It is advisable to wear cool loose-fitting clothing and keep your weight down to minimize friction. Avoid sweating and friction by doing exercises such as swimming, rather than activities such as cycling. Holidays in hot and humid countries may make the condition worse.