ConditionSkin, hair & nails
Hailey-Hailey Disease (Familial Benign Pemphigus)
Hailey-Hailey disease is a rare inherited skin condition in which skin cells do not stick together properly, so friction and sweat cause blisters and raw, cracked, often smelly patches in skin folds such as the armpits, groin, neck and under the breasts. It usually starts in the teens to 30s and comes and goes in flares, worse in summer. It is not contagious and is not true pemphigus. Keeping folds cool and dry, treating infection and anti-inflammatory creams control most flares.
Also called: Familial benign chronic pemphigus, benign familial pemphigus, HHD
At a glance
- What it is
- An inherited blistering skin disease affecting skin folds.
- Cause
- Changes in the ATP2C1 gene, inherited in an autosomal dominant way.
- Symptoms
- Small blisters, raw cracked plaques ("dried riverbed" look), itching, pain, odor.
- Triggers
- Heat, sweat, friction, tight clothing, infection, stress.
- Treatment
- Cool, dry skin care, topical steroids and antimicrobials, and procedures for stubborn areas.
When to get help
See a doctor within a day or two
- Rapidly spreading painful blisters or punched-out sores, especially with fever (possible herpes infection on top of the condition)
- Spreading redness, pus, or fever with a flare (possible bacterial infection)
Book a routine appointment
- Recurrent blisters or raw, cracked patches in the armpits, groin or neck
- Flares that are not controlled by your usual treatment
- Painful cracks that make walking or moving the arms difficult
- Wanting to discuss procedures or family planning
Usually safe to manage at home
- A mild flare in hot weather that improves with cool showers, loose cotton clothing and your prescribed cream
What causes Hailey-Hailey disease
The condition is caused by changes (mutations) in the ATP2C1 gene, which controls calcium inside skin cells. Calcium is needed for the "glue" (desmosomes) that holds skin cells together. With less glue, the outer skin cells separate under stress, a process called acantholysis, forming blisters and erosions.
It is inherited in an autosomal dominant pattern: each child of an affected parent has a 1 in 2 chance of inheriting it. Some people have no family history because the change arose new in them. Severity varies widely, even within families.
Symptoms
- clusters of small, fragile blisters that break easily
- red, raw, moist patches with crusting
- cracks and fissures in a pattern that has been compared to a dried riverbed
- itching, burning and pain
- an unpleasant odor from bacteria and yeast in damaged skin
- darkening of the skin after flares
Where it appears
- armpits
- groin and inner thighs
- sides and back of the neck
- under the breasts
- around the anus
- less often, the trunk and elbow creases
Nails
Some people have white lines running along the length of the fingernails, a helpful diagnostic clue.
Course
Symptoms usually appear between the late teens and 40s, though they may start earlier or later. The disease is chronic, with flares and quieter periods. Flares are often worse in summer. Some people find it improves with age.
Triggers
- heat and humidity
- sweating
- friction from tight clothing, bra straps and exercise
- skin infections, including bacteria, yeast and herpes simplex
- sunburn
- emotional stress
- in some women, the menstrual cycle or pregnancy
Look-alike conditions
| Condition | How it differs |
|---|---|
| Intertrigo | Red rash in folds from moisture and friction, without blisters or family history |
| Inverse psoriasis | Smooth, shiny red patches in folds, often psoriasis elsewhere |
| Fungal or yeast infection | Itchy red rash with satellite spots, responds to antifungals |
| Hidradenitis suppurativa | Painful lumps, abscesses and tunnels, not blisters |
| Darier disease | Related genetic condition with greasy, warty bumps, often on chest and back |
| Pemphigus | Autoimmune blistering disease, often with mouth sores, more widespread |
Diagnosis
A dermatologist suspects Hailey-Hailey disease from the appearance, location and family history. A skin biopsy confirms it, showing skin cells separating in a typical "dilapidated brick wall" pattern, and negative tests for the antibodies seen in pemphigus. Swabs check for bacteria, yeast or herpes during flares. Genetic testing can identify the ATP2C1 change but is not always needed.
Treatment
There is no cure, but treatment controls flares and reduces how often they happen.
Daily care
- keep skin folds cool and dry: cool showers, fans or air conditioning, and drying thoroughly
- wear loose, breathable cotton clothing, and avoid tight waistbands and straps
- lose weight if skin folds are deep
- use antiperspirant if tolerated, or consider treatments that reduce sweating
- wash gently with antiseptic or mild cleansers
Medicines
- topical steroids to calm inflammation during flares
- topical calcineurin inhibitors (tacrolimus, pimecrolimus) as steroid-sparing options
- topical or oral antibiotics and antifungals for secondary infection, which is a common flare trigger
- antiviral medicine for herpes infection
- oral medicines for severe disease, such as doxycycline, retinoids (acitretin), low-dose naltrexone (reported helpful in some people) and others used off-label
Procedures
- botulinum toxin injections reduce sweating in folds and can reduce flares
- laser resurfacing, dermabrasion or photodynamic therapy can replace affected skin with more resistant skin in stubborn areas
- surgical removal and grafting of severely affected skin, rarely
Outlook
Hailey-Hailey disease is lifelong but not life-threatening and does not affect internal organs. With good skin care and prompt treatment of flares and infections, most people manage it well. The condition can affect comfort, self-image and intimacy, and support from a dermatologist and patient groups helps.
Frequently asked questions
What triggers Hailey-Hailey flares?
Common triggers are heat, sweating, friction from clothing or exercise, skin infections (bacterial, yeast or herpes), sunburn and stress. Keeping skin folds cool and dry and wearing loose cotton clothing reduces flares.
Is Hailey-Hailey disease contagious?
No. It is a genetic condition and cannot spread from person to person. However, the damaged skin often becomes infected, and those infections, such as herpes, can be contagious.
Is Hailey-Hailey disease the same as pemphigus?
No. Despite the name familial benign pemphigus, it is a genetic disorder of skin cell adhesion, not an autoimmune disease like pemphigus vulgaris. It mainly affects skin folds, rarely involves the mouth, and is not life-threatening.
Can Hailey-Hailey disease be cured?
There is no cure yet because it is genetic, but treatment controls symptoms. Topical steroids, antimicrobials, botulinum toxin and laser or dermabrasion for stubborn areas help many people achieve long periods of clear skin.
Sources
This guide was written from current patient and clinical guidance, including:
- Genetic and Rare Diseases Information Center (NIH): Hailey-Hailey disease
- MedlinePlus Genetics: Hailey-Hailey disease
- British Association of Dermatologists: Hailey-Hailey disease patient information leaflet
- DermNet: Hailey-Hailey disease
- American Osteopathic College of Dermatology: Hailey-Hailey disease