Hailey-Hailey Disease

What is Hailey-Hailey disease?

Also known as Familial Benign Chronic Pemphigus or Gougerot-Hailey-Hailey Syndrome, this is a distinct genetic disorder caused by a gene mutation. It seems that this disease is extremely rare, being hereditary transmitted. First identified and described in 1939, this blistering skin disease usually becomes obvious after puberty but it can occur at any age. The skin will present blisters and erosion, the main areas affected being the neck, underarms, skin folds and genital area.It is important to understand that this disease occurs due to a mutation in a specific gene and it should not be confused with pemphigus. The latter is an autoimmune disorder, in which the body practically attacks itself, producing auto-antibodies. Hailey Hailey disease is not an autoimmune disorder, the body does not attack itself, nor does it produce auto-antibodies. It is a genetic disorder and the gene mutation is responsible for all the symptoms.

Symptoms of Hailey-Hailey Disease

Before the symptoms of the Hailey Hailey disease are presented, it is important to understand that there are factors contributing directly to the exacerbation of the symptoms, such as excessive heat, prolonged sweating and constant friction. As a matter of fact, most patients complain of the disease becoming worse during hot summer days, because of the obvious reasons.These are the most common symptoms of Hailey Hailey disease:

• Initial debut - skin rash accompanied by pain and erosion, the typical place for appearance is any skin fold in the body.
• Rashes and blisters in any of the following areas of the body: neck, underarms, skin folds, genital area, under the breasts, between the buttocks.
• Lesions appear and disappear, leaving no scars in the meantime.
• In case a lesion extends, under a careful analysis, one will see that the middle of the lesion becomes clear, while the border will be more pronounced, taking a typical round shape.
• Prolonged lesions tend to become engorged and, as the process further develops, the skin will become deteriorated and the emerging cracks will be quite painful.
• Often times, the disease is complicated by an additional infection, bacterial, fungal or viral, leading to excessive body odor.
• Additional symptoms include the appearance of white bands on fingernails and cracks in the palm.

Causes of Hailey-Hailey Disease

Even though Hailey Hailey disease is hereditary, there have been some cases identified in which no medical history was present. According to the medical literature, the main cause that leads to the appearance of this disease is a gene mutation. The guilty gene is ATP2C1, commonly found on the third chromosome and it is the gene commonly responsible for the proper functioning of a calcium and manganese pump. If that gene does not do its job, then the skin cell do not stick together and they are not properly assembled as well. It would be just as if you would try to build a wall without any cement; the bricks wouldn't stick together and the same happens with skin cells in Hailey Hailey disease.Because of the gene mutation, the skin cells lack the necessary protein quantity in order to stick together and this malfunctions leads to the appearance of blisters and rashes. Under a microscope, one can notice how the cellular interconnections are lost and the skin lesions formed. The fact that Hailey Hailey disease is dominantly inherited means that there is a 50% chance that each child of an affected parent will inherit the skin condition.

Treatment

As there is no diagnostic test for Hailey Hailey disease, there can be no talking of a definite cure. There are preventive measures you can take in order to prevent the exacerbation of the disease and available treatments for reducing the existing symptoms.These are the several prevention measures you can consider:

• Excessive heat, prolonged sweating and constant friction represent three of the major trigger factors for Hailey Hailey disease.
• Avoiding them is a good prevention method. Also, you might want to protect yourself against sunburns, as this can leave the skin in a pretty frail state
• Excessive sweating can be reduced by wearing special wads in your underwear but also by wearing clothes that are made 100% cotton. Avoiding hot, humid temperatures might also be a good idea
• Maintain perfect bodily hygiene. It goes without saying that a secondary infection, be it bacterial or fungal, will only complicate the skin lesions. Use special soaps and make sure you clean all the affected areas in a correct manner
• Wet compresses can calm the skin

These are treatments aimed at reducing existing symptoms:

• Hydro-cortisone creams - this topical treatment can help you not only with the calming of skin lesions but it can also guarantee faster healing. However, you should take it only for the recommended period of time, otherwise it can have serious side effects.
• In case of secondary infections - anti-fungal or anti-bacterial topical creams or medication will help you with the additional symptoms, preventing the lesions from becoming worse or extended.
• Anti-viral medication administered orally is recommended in case of secondary viral infections, such as the one caused by the presence of herpes simplex in the body.
• Oral antibiotics are recommended for secondary bacterial infections. Be sure to take them for as long as they are prescribed or you might risk not killing the bacteria, but rather make it stronger. As it was already mentioned, you can fight bacteria with anti-bacterial creams as well, especially those that are based on benzoyl peroxide.
• For painful skin cracks, one of the best topical creams that is recommended is Calcipotriol, as it contains a synthetic derivative of vitamin D and it can help with the faster healing of these cracks.
• Oral retinoids - use them only at the recommendation of a doctor and only for a short period of time, as these can have serious side effects.
• Anti-sweating powder and botulinum toxin are recommended for excessive sweating, the latter one having only reduced effect (plus, you need to repeat the toxin shot every couple of months).
• Just as with other skin conditions, you can consider PUVA photo-therapy for the alleviation of upsetting symptoms.

Is Hailey Hailey disease contagious?

The answer is simply no. It is a genetic disorder that is transmitted from parent to offspring and you do not have to worry about it being transmitted to other people, in other ways. Hailey Hailey disease is not contagious.

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