Hailey-Hailey disease

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1. Overview

Hailey-Hailey disease is also known as familial benign chronic pemphigus.

It is a rare skin disease, inherited, characterized by the appearance of areas covered by reddish scabs or small blisters, bullous, after friction.

Those who suffer from this disease consider it annoyance rather than a serious problem.


1. Overview
2. Causes
3. Symptoms
4. Diagnosis
5. Treatment
6. Home self-care

2. Causes

A small error in the DNA code of a gene on chromosome number 3 (one of the 46 human chromosomes) have been shown to cause this condition. Normally, the gene plays an important role in linking corresponding cells of the outer layer of skin (epidermis).

In Hailey-Hailey disease case, the adhesion of cells is damaged, the cells from epidermis being separated especially in skin areas prone to friction, such as the groin and under the arms. Abnormal gene is genetically transmitted in both women and men.

Familial benign chronic pemphigus is not a contagious disease or the result of allergies. There is a tendency for the disease to worsen when the areas prone to friction sweat, especially in hot weather.

The condition is dominant inherited, which means there is a 50% chance that each child that has an affected parent to inherit the problem.

3. Symptoms

Hailey-Hailey disease is unpleasant rather than serious. Even if the affected skin is itchy and crusty, these manifestations come and go. There may be a discomfort to the affected skin where skin surfaces rub for a long period of time, such as walking situation.

Very rarely is a worsening of symptoms in the genital area or crease of the buttocks, which can make walking very uncomfortable.

The first signs of the condition usually occur in people aged between 15 and 40 years, but it can also occur outside this age group. Severity varies enormously and the events are unpredictable. Erythematous scaly red areas or small vesicles occur in areas of friction, especially in the sides of the throat or skin folds under the arms, upper legs or under the breasts.

Skin looks normal between the episodes in which the disease manifests and don not cicatrizes. Many patients find that their condition improves as they get older.

4. Diagnosis

Condition is often confused with eczema or contact dermatitis (determined by an allergy), a rash caused by sweat, ringworm, impetigo (bacterial infection of the skin) or fungal infection. Hailey-Hailey disease diagnosis can be established by examining a sample of skin previously collected (biopsy) under the microscope.

5. Treatment

There are several ways to relieve the symptoms of Hailey-Hailey disease. One alternative is cream containing cortisone. This is very useful and can be prescribed in a mixture with an antibiotic to prevent infection of the skin.

To topically applied cortisone ointments may appear a sensation of stinging or itching. Cortisone cream can prevent symptoms amplification if applied immediately after the itchy sensation appears. Ointment should be applied in a very thin layer, just once or twice daily on the affected skin. Do not put any cream on the affected skin, excepting that prescribed by your doctor.

The affected skin may smell unpleasant especially in wet areas. The smell is typical of this condition and does not mean that the skin is dirty. Bacteria or fungi accentuate the itching sensation or may even be the trigger. Carefully washing is very important and will use the bathroom antiseptic, antiseptic creams, antibacterial or antifungal soaps. They may also be useful orally, administered as antibiotics.

Hailey-Hailey disease may increase the risk of developing skin infections. Infection with the same type of bacteria that are responsible for infection of cuts and scrapes is common and can worsen the familial benign chronic pemphigus. This will require antibiotics prescribed by a doctor.

Herpes simplex virus of the skin can sometimes cause infections in people who suffer from Hailey-Hailey disease. If the patient’s general health gets worse and the pain gets worse, this may be a sign of infection with this virus. In this case you should contact your GP or dermatologist because you may need antiviral medication.

Recently it was found that topical application of tacrolimus cream succeeded to improve the symptoms of this disease in a significant number of patients, although this preparation has not yet been approved for the treatment of Hailey-Hailey disease. In addition to these, treatments that affect the immune system (eg corticosteroids, dapsone, cyclosporine or methotrexate) may occasionally be used. However, potential effects are serious and are used only in patients who do not respond to less severe treatment.

It was observed that topical photodynamic therapy was very helpful in curing this condition, in some cases, but not in the resistant one. This involves applying a cream that causes sensitization of the cells that will be exposed to light, followed by exposure to a special red light lamp, three hours later. Treatment may be painful.

Other possible approaches to treatment are still under evaluation and include the use of botulinum toxin (to reduce perspiration), surgical removal of the affected areas (which can lead to scar formation) and laser treatment.

6. Home self-care

You should avoid all the triggers that can cause these stains: heat, sweating and friction. For example, if you buy a new shirt and you do not want it to scratch the skin, will soften collar before wearing.

It is advisable to avoid wearing tight clothing. It is recommended to wear comfortable clothes and avoid transportation difficulties to reduce friction. Also, avoid sports resulting in much sweat, preferring instead sports like swimming, for example. You should take into account that high temperatures and wet weather may aggravate the disease.



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