MENU
Therapeutics in Dermatology
A reference textbook in dermatology

Hailey-Hailey disease (Benign familial pemphigus)

30 July 2019, by MENDES R.

1 - ACKNOWLEDGEMENTS

Chapter written with the help of the EADV, the Fondation René Touraine and the Therapeutics in Dermatology

2 - OVERVIEW

Hailey-Hailey disease, also known as familial benign chronic pemphigus, is a rare inherited skin disorder. It is characterized by a painful rash and blistering in areas of friction, such as the skin folds. The disease flares intermittently and lesions tends to come and go. Heat, friction and sweating aggravate the lesions and pain may limit physical activities. Secondary bacterial, fungal or viral infections are common and may also exacerbate a flare-up. The disorder usually becomes apparent in the third or fourth decade and tends to persist lifelong. In most cases, there is a family history of the condition.

3 - SYMPTOMS

Hailey-Hailey disease presents after puberty, usually between the ages of 15 and 40 years, but it may begin at any age. The symptoms and severity of the disease differ from one person to another, even among members of the same family. Red scaly patches, superficial blisters and erosions appear at areas of friction, especially at the groin, armpits, neck, under the breasts and between the buttocks. Itching and burning sensation are the common symptoms. In some cases, pain may limit physical activities. Secondary bacterial, fungal or viral infections of the skin lesions can also occur and may cause an unpleasant odor.

The lesions recur periodically and can resolve spontaneously without scarring. The length of remission varies widely. Sunlight, heat, sweating and friction often aggravate the disease.

4 - CAUSES

Hailey-Hailey disease is an inherited skin condition, although occasionally sporadic cases arise without a family history. It is caused by a genetic change (mutation) in the ATP2C1 gene. This gene plays an important role in ensuring that the cells in the outer layer of the skin (the epidermis) stick together. In Hailey-Hailey disease this adhesion of cells is weakened, resulting in separation of the cells from one another, especially in areas of skin prone to friction such as the groin and under the arms.

5 - TREATMENT

There is currently no cure for Hailey-Hailey disease, but treatment can help keep the symptoms under control. Management depends on the severity and extent of the condition. General approach includes careful hygiene and washing, avoiding triggers and reduction of sweating. Prevention and treatment of bacterial, viral and fungal infections is also important, and drugs used to treat these infections are commonly used.

Corticosteroid creams are effective and may be prescribed in combination with topical antibiotics or antifungals. Alternative topical treatments include tacrolimus and calcitriol. More severe cases may require oral antibiotics (especially erythromycin and tetracycline), oral corticosteroids, oral retinoids and other treatments that affect the immune system (e.g. ciclosporin, dapsone or methotrexate).

Botulinum toxin reduces sweat production and has been used for Hailey-Hailey disease. Other possible therapies include photodynamic therapy (a type of light treatment), laser treatment and surgery.

Genetic counselling is recommended for affected individuals and their families.

6 - TIPS FOR MANAGING

There are also general measures that may help you prevent outbreaks:

  • Avoid trigger factors such as heat, sweating and friction
  • Wash with an antiseptic soap substitute or antiseptic bath oil
  • Dry skin folds carefully
  • Wear cool, soft and loose clothing
  • Keep your weight down
  • Avoid exercises that cause friction (e.g. cycling)
  • Apply wet compresses to dry up oozing patches
  • Apply moisturizing creams
  • Use antiperspirants of aluminium salts
  • Use sunscreen

× N.B. : This limited content is for the general public. If you are a health professional, click here to register for free and gain access to a dedicated deeper content.
If you already have an account, log in!

Follow us

Newsletter

  Health professionals