Hailey-Hailey disease is a relatively uncommon disease in dermatology, but we have had several inquiries from patients with Hailey-Hailey disease in the last two days, so we would like to write a short article in the simplest language to help more patients.
What is Hailey-Hailey disease?
The Hailey brothers, both doctors, discovered a very strange skin disease together in 1939, so they named this strange skin disease after them internationally, called Hailey-Hailey disease (Hailey-Hailey disease).Hailey-Hailey disease is called chronic familial benign aspergillosis in Chinese (abbreviated as The Chinese word for Hailey-Hailey disease is chronic familial benign aspergillosis (“slow familial liang”), also called familial benign chronic aspergillosis.
What are the problems that occur with the skin when you have this disease?
The disease behaves just like its Chinese name – chronic familial benign aspergillosis.
”Slow” – chronic: this is a disease that will exist for a long time, and once you have it, it is not something that can be cured quickly, and may accompany the patient for the rest of his or her life, repeatedly, very annoying.
”Family” – familial: the patient is often not alone in the battle, many relatives in the family may have this problem, and these relatives are concentrated on one side of the father or mother. Of course this is not absolute, there are also about 30% of patients who have normal family members.
”Good” – benign: having this disease is not life-threatening, except that it tends to recur and can be very, very distressing to the patient’s quality of life when it flares up.
”Pemphigus”: the skin will repeatedly blister, vesicular surface, and crack. In mild cases, the blisters may only grow in a certain area, such as in the armpits or groin; in severe cases, the blisters may appear in a wide range of areas, including the armpits, groin, under the breasts, neck, elbow fossa, popliteal fossa, perianal area, and other places that are prone to friction. When you have blisters, you will also have itching, odor, local erosion, cracking, and pain when you rub.
How can I be diagnosed with this disease?
There are many diseases that have similar skin manifestations, such as eczema, transitional psoriasis, follicular keratosis, proliferative aspergillosis, ringworm, and even a cancer called extramammary Paget’s disease, which can also have these symptoms. .
It is important to go to a regular hospital and often a skin biopsy and histopathological and genetic tests are needed to make a definitive diagnosis.
Skin biopsy and histopathological examination are performed by cutting off a piece of skin the size of a soybean after anesthesia is applied to a certain area of skin and sending it to a laboratory to be sectioned for observation and diagnosis under a microscope by a professional doctor.
For the genetic examination, the blood of the patient and family members is drawn, about 2ml is enough, and then through a series of experiments, the genes are analyzed to find out which gene has been changed in which position, causing the disease.
Why do I have this disease?
The disease is inborn.
Some patients may object, saying, “I was born fine, and I didn’t have this problem at all when I was a child!” It is true that the disease is present at birth, but it does not manifest itself as a child, and the symptoms do not begin to appear until after puberty, or even in the 30s or 40s.
Can my child get this disease?
Hailey-Hailey disease is an autosomal dominant genetic disorder. Humans have about 30,000 genes that control our life, illness and death. One of these genes, called ATP2C1, can go wrong and cause Hailey-Hailey disease, and it is possible to pass this problematic gene on to offspring.
In most cases, if only one parent has the disease and the other parent is normal, the genotypes of the parents are Aa and aa, respectively, and the child has a 50% chance of having the disease. If the child is only a few years old, it is difficult to determine whether the child has the disease by looking at the child’s behavior alone, unless genetic tests are done.
How can I stop my child and future generations from getting the disease?
With the current medical technology, there is no way to change the genes of a child that has already been born, so we can only hope that he or she will not get the disease. If the child does develop the disease, there is no need to be upset, as there are many ways to control the symptoms.
If parents do not want their offspring to have the disease again, they can consider prenatal genetic testing or third-generation IVF to select a normal baby who will theoretically have no more offspring with the disease.
Is Hailey-Hailey disease contagious?
No, it is not contagious, it is not contagious! It is important to say this three times.
Although Hailey-Hailey disease may look wet and dirty under the armpits and thighs, it is not contagious to family and friends, either by direct contact with the patient or by touching the patient’s clothing.
Can Hailey-Hailey disease be cured?
This is a question that every patient asks, but unfortunately, Hailey-Hailey disease is caused by a congenital genetic problem and there is no fundamental cure.
What are the best treatments for Hailey-Hailey disease?
There are many treatment options, divided into topical treatments and systemic treatments.
Among the traditional topical treatment modalities, topical glucocorticoids (e.g. Eloson, Piperisone, Halometasone, etc.) and antibiotic creams (e.g. Fusidic acid, Bactrim, Erythromycin ointment, etc.) are very effective for the majority of patients with Hailey-Hailey disease, and can also be applied wet on the affected area with hormonal or astringent drug solutions. Topical tacrolimus ointment has also been effective in some patients.
Systemic treatment is commonly used with oral tetracyclines and erythromycin, which, in addition to being antibacterial, have the effect of reducing epidermal relaxation. In severe cases, oral hormones and retinoids (e.g., Tylenol, Avelox) can also be considered. This is coupled with a variety of laser treatments or photodynamic therapy. In some cases, oral immunosuppressants may also be considered.
Among the new treatment modalities, two are worth introducing to you.
1, Botox injection: pain index ☆☆☆, post-operative recovery time ☆, price ☆☆☆☆☆, safety ☆☆☆☆, maintenance time ☆☆☆☆, efficacy ☆☆☆☆, recurrence rate ☆☆☆☆
Botulinum toxin injection is a very mature cosmetic treatment technique, which is highly sought after by beauty seekers worldwide because of its efficacy and safety, mainly for removing facial wrinkles, slimming the face, slimming calves, shrinking pores and controlling oil, excessive underarm sweating, armpit odor, hand sweating and so on. However, several international studies have found that Botox injections are also very effective in treating Hailey-Hailey disease. The downside is that the price is high, the maintenance time is not long enough, and the injections need to be repeated every six months or so.
If patients with Hailey-Hailey disease have stubborn lesions and have used other medications with poor results, they may consider trying Botox injections.
2.Grinding: pain index ☆☆☆☆, post-operative recovery time ☆☆☆☆, price ☆☆☆☆☆, safety ☆☆☆☆☆, maintenance time ☆☆☆☆☆, efficacy ☆☆☆☆, recurrence rate ☆☆☆
It is possible to mechanically grind or laser grind to remove the lesioned epidermis and to allow the skin to repair again. It is suitable for patients with Hailey-Hailey disease who have hypertrophic lesions and whose condition is stubborn and difficult to treat. The advantage is that the treatment effect is good and the recurrence rate is low, and some patients can be treated once without recurrence for several years or even never again. However, the treatment process is relatively painful and the recovery time is long.
It should be noted that fractional laser grinding, which has become more popular in recent years, is very effective in the field of skin rejuvenation and cosmetic scar removal, but since this method can only partially grind, I do not think it is suitable for the treatment of Hailey-Hailey disease.
What should Hailey-Hailey patients pay attention to in their lives?
Good lifestyle habits can greatly reduce the number of episodes of Hailey-Hailey disease, as well as improve the severity of symptoms.
Sweating and friction can aggravate lesions, so two important things to do to reduce symptoms are to reduce sweating and to reduce friction.
For example, it is important to stay indoors and in an air-conditioned environment as much as possible, and to pay attention to skin cleanliness and hygiene; wear more loose cotton clothing, especially underwear. In addition, reduce weight, can also reduce the friction between the skin, also need to avoid local friction serious sports, such as long-distance running, soccer, etc..