Hailey-Hailey Disease

Overview

Hailey-Hailey disease, also called familial benign chronic pemphigus, is a rare inherited skin condition in which the cells of the upper skin layer do not stick together properly. This causes the skin to break down easily in areas of friction, producing recurring blisters, raw red patches, and painful cracks, mainly in the folds of the body.

The disorder usually begins in the teens to forties and tends to flare and settle over many years. Despite the word "pemphigus" in its older name, it is not an autoimmune disease; it is caused by a genetic change. "Benign" refers to the fact that it is not life-threatening, although flares can be uncomfortable and disruptive. With careful management, most people can reduce how often and how severely it flares.

Symptoms

Symptoms come and go, with flares often triggered by heat, sweat, and friction. The most affected sites are the sides of the neck, the armpits, the groin, and the skin under the breasts.

• Red, raw, moist patches in skin folds
• Small blisters that break easily, leaving crusted or weeping areas
• Painful cracks (fissures), especially in the depth of a fold
• Itching, burning, or soreness in the affected skin
• An unpleasant odor when areas become secondarily infected
• White lengthwise streaks on the fingernails in some people

Flares tend to be worse in hot weather and during physical activity, and the same areas are often affected repeatedly.

Causes

Hailey-Hailey disease is caused by a mutation in a gene called ATP2C1. This gene helps skin cells manage calcium, which they need to stick firmly to one another. When the gene is faulty, the connections between skin cells in the upper layer are weak, so the skin separates and breaks down under friction and moisture.

The condition is inherited in an autosomal dominant pattern, meaning a person needs only one copy of the altered gene, usually from one affected parent, to develop it. About half of the children of an affected parent inherit the condition, though severity varies widely even within the same family.

Risk Factors and Triggers

The main risk factor is having a parent with the condition. Within affected individuals, certain factors trigger or worsen flares:

• Heat and sweating
• Friction from skin rubbing together or from clothing
• Skin infections with bacteria, yeast, or the herpes virus
• Sunburn or other skin injury
• Stress

Diagnosis

A diagnosis is based on the typical pattern of recurring fold rashes, a family history, and skin testing.

• Skin examination and history: Recurrent raw patches and blisters in the neck, armpits, and groin, often with affected relatives.
• Skin biopsy: A small sample examined under the microscope shows the characteristic separation of skin cells, which confirms the diagnosis.
• Swabs or cultures: Used to check for secondary bacterial, yeast, or viral infection during flares.
• Genetic testing: Occasionally used to confirm the ATP2C1 mutation in uncertain cases.

Treatment

There is no cure, so treatment focuses on calming flares, treating infection, and reducing the friction and moisture that trigger the skin breakdown.

• Topical steroids: Corticosteroid creams reduce inflammation during flares.
• Topical antibiotics or antifungals: Used to treat or prevent the bacterial and yeast infections that often complicate flares.
• Oral antibiotics or antivirals: Prescribed for more significant infections, including herpes infection of the affected skin.
• Other therapies: For stubborn disease, dermatologists may use treatments such as topical calcineurin inhibitors, laser therapy, botulinum toxin to reduce sweating, or other specialist options.
• Keeping skin cool and dry: A cornerstone of management to limit flares.

Prevention and Self-Care

• Keep affected folds cool and dry; use absorbent powders and barrier creams
• Wear loose, breathable clothing and avoid overheating
• Reduce friction with soft fabrics and, where helpful, weight management
• Treat skin infections promptly to prevent them triggering flares
• Use gentle, non-irritating skin cleansers
• Manage stress and protect skin from sunburn and injury

When to See a Doctor

See a dermatologist if you have recurring raw, blistering patches in skin folds, particularly if relatives have had similar skin problems, so the diagnosis can be confirmed and a long-term plan made. Seek care during a flare if the skin becomes increasingly painful, foul-smelling, crusted, or starts spreading rapidly, as these can signal a bacterial, yeast, or herpes infection that needs specific treatment. Ongoing follow-up helps tailor therapy as the condition changes over time.

Related Topics

Frequently Asked Questions

References

1. National Organization for Rare Disorders (NORD). Hailey-Hailey Disease.
2. Genetic and Rare Diseases Information Center (GARD). Hailey-Hailey disease.
3. DermNet. Hailey-Hailey disease.
4. MedlinePlus, U.S. National Library of Medicine. Benign familial pemphigus.