Hailey-Hailey Disease and Current Treatment

By: Khushi Sheth

Image Credit: Flickr @ Adrian Scottow

Hailey-Hailey Disease is an extremely rare inherited disorder that results in itchy, sore, and red scaly patches of skin. What may look like a normal rash could in fact lead to skin erosion, or the loss of the epidermis, as well as superficial blisters in those with Hailey-Hailey disease. The main areas on the skin where these blisters form include the groin, armpits, neck, and under the breast, essentially places where the skin folds and there are higher amounts of friction. Depending on the person, the symptoms of this disease can come and go with varying severity although most people with Hailey-Hailey disease are able to live normal lives.

Genetically, this condition arises due to a mutation on chromosome number 3. This gene is responsible for building the cells of the epidermis (the top layer of skin). As mentioned before, the mutation causes a delay in the growth of the cells of the epidermis. Physically, in Hailey-Hailey disease, the cells of the epidermis do not properly adhere to each other, which results in skin weakness and the primary symptoms of Hailey-Hailey.


One factor that leads to skin erosion and flare-ups is friction. Because the groin and armpits are most prone to friction, those are the areas where skin eroding would most likely occur. Aside from friction, the severity is also impacted by factors such as sweat and hot weather.


Unfortunately, there is no cure for the disease, as scientists are not able to revert the genetic defect back to its correct form. But there are temporary treatments that help with the varying periods of severity. A corticosteroid anti-inflammatory cream can help prevent rashes from spreading when they begin to develop. Patients with the Hailey-Hailey disease are more likely to develop infections, due to their exposed sores. Because areas of flare-up are very sensitive and prone to infection, carefully washing them with antiseptic products helps to prevent infection. If bacterial infections occur, they may be treated with oral antibiotics, while viral infections (such as those caused by the Herpes simplex virus) require a special antiviral drug. For especially severe or resistant cases of Hailey Hailey, one more intensive and painful approach is called topical photodynamic therapy, which exposes the skin to light after applying a special cream that sensitizes them. Other extreme treatments include laser surgery and surgery to reduce the amount of sweat produced from the glands, which can then reduce the number of flare-ups a person experiences.


Educational Content

Q: Who can primarily get Hailey-Hailey Disease?

A: Both men and women are equally at risk of receiving the disease. This condition is primarily autosomal dominant, meaning that there is a fifty percent chance of inheriting the trait for Hailey-Hailey. Autosomal dominant is a pattern of inheritance for a gene that is located on non-sex chromosomes and just one copy of the disease-associated mutation is enough to cause the disease in the offspring.


Q: Because this disease is so rare, how is Hailey-Hailey disease diagnosed?


A: Although Hailey-Hailey can easily be mistaken for eczema, a direct immunofluorescence test on a skin sample can confirm the disease. This is a method in which antibodies are labeled with fluorescent dyes in order to visualize the molecules and movement of what could be Hailey-Hailey. This disease is not curable but there are plenty of treatable options.


Citations

  1. https://knowyourskin.britishskinfoundation.org.uk/condition/hailey-hailey-disease/#:~:text=Hailey-Hailey%20disease%20is%20also%20known%20as%20familial%20benign,the%20groin%2C%20armpits%2C%20neck%20and%20under%20the%20breasts.

  2. https://www.medicinenet.com/hailey-hailey_disease_familial_benign_pemphigus/article.htm

  3. https://rarediseases.info.nih.gov/diseases/6559/hailey-hailey-disease

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