Introduction

Definition of hailey-hailey disease

Hailey-Hailey disease (HHD), also known as Familial benign pemphigus, is a rare genetic skin condition that causes blisters and erosions in different areas, such as the neck, skinfolds, armpit and genitals. It is caused by a mutation in the ATP2C1 gene, which causes the skin cells to not stick together, leading to the breaking of skin layers, resulting in painful blisters and erosions. 

Since there is no cure for HHD, the only way is to prevent the flares from occurring, to improve your quality of life, as the flares are triggered by environmental, mechanical and other factors.^1,2

Understanding flares in hailey-hailey disease²

Symptoms

The symptoms for HHD may vary from person to person, but in most cases, there is always a family history of the disorder. 

HHD first shows a blistering, erosive skin rash that mostly affects the armpits, chest, neck and groin. These blisters can then develop into a yellow, crusty overlying layer. It can also be itchy and may also burn, leaving painful, cracked skin. 

The skin blisters are generally remitting and relapsing and go away on their own, but they can also recur from time to time. 

Triggers of flares

The disorder is triggered by humidity, friction, heat, trauma and secondary bacterial, fungal infections. It can also be worsened by wearing tight clothing and excessive physical activity. 

Daily skin care for flare prevention^2,3

There are many steps you can add to your daily life that can help prevent the flares, including:

• Using mild and fragrance-free soaps and cleansers for the skin
• Regular moisturisation     
• Avoid hot showers and harsh scrubbing
• Using protective creams - zinc oxide, petroleum jelly on areas that are prone to friction.
• Wearing soft, comfortable and breathable clothing 
• Using organic antiperspirants in non-affected areas
• Applying absorbent powders to keep the skin dry

Medical treatments for flare prevention^3,4

Even though HHD cannot be cured, there are various ways and techniques that help to prevent flares, including:

• Corticosteroids - used for short-term flare control.
• Calcineurin inhibitors - tacrolimus or pimecrolimus to reduce inflammation 
• Antifungal and antibacterial creams - to prevent secondary infections
• Oral antibiotics and antifungals - for flare prevention in current infections
• Laser treatments - to reduce recurrent blisters 
• Surgical intervention, in severe cases, is required to remove affected skin areas

Lifestyle modifications for flare prevention^{2, 5}

To improve better quality of life, you can:

• Practice techniques such as mindfulness, relaxation exercises, or therapy to manage stress
• Avoiding food allergens that may contribute to skin irritation.
• Having a balanced diet to support skin health
• Regular low-friction exercises - swimming, cycling
• Using protective clothing and taking measures to reduce sweating.

Future research and emerging therapies 

Emerging therapies for HHD include the use of oral anticholinergic medications, such as glycopyrrolate and naltrexone. Glycopyrrolate is thought to control sweating. Whereas naltrexone is used because it blocks receptors, but should only be used at low doses.⁶

Conclusion

HHD is a rare chronic genetic skin disorder that causes skin blisters provoked by friction, humidity and heat. There may be ways to prevent the painful blisters, such as topical creams and other medical creams, such as corticosteroids. Currently, there is no cure for HHD. New research suggests a new medication which has shown promising results in controlling HHD.