Hailey-Hailey Disease

Hailey-Hailey disease also called as familial benign chronic pemphigus is a rare chronic autosomal dominant disorder of skin. The name comes from the Hailey brothers who first described in 1939. It is characterized by recurrent blistering, crusting and erosion in the skin fold areas like groin, neck folds, chest, under the breasts and axillary areas.

Haily-Haily disease is most common on late twenties to forties but may occur at any age. The main cause is unknown but its is thought to be due to genetic defect in calcium pump protein. Besides genetic connection there are other contributing factors that might exacerbate it. Some factors that might exacerbate the disease are heat, sweating, friction, physical trauma , stress and infection. Most people often find their symptoms worsening during the summer.

Initially burning and itching followed by  red, scaly or small fluid filled blister may appear on area where most friction occurs. The blisters may then rupture with malodorous in case of secondary infection. The lesion may be localized in certain area or sometimes generalized. These lesion comes and heals frequently without leaving scars. Patients with Hailey Hailey disease may often present with nail manifestation which looks like longitudinal white bands in the fingernails but are not brittle or fragile. Some females may also find their symptoms worsen during premenstrual periods. This suggest that Hailey-Hailey disease may have some connection with sex hormones.

Your doctor may diagnosis this Disease by its appearance and family history and confirm it with skin biopsy. This disease can sometimes be mistaken for fungal infection, heat rash, bacterial infection, eczema and other blistering conditions, so skin biopsy is highly recommended in case of suspicion.

There is no cure for Hailey-Hailey disease but its not a life threatening disease. The aim of treatment is to avoid aggravating factors like sweating, friction, stress and infection. You should avoid tight clothes, wear light loose clothes, avoid excessive sweating , keep the body folds cool and dry. Sunscreens might help to some extend.  There are various treatments options that have been tried. Local lesions may be treated with topical corticosteroids and topical antibiotics. For widespread cases topical treatment with oral antibiotics may be given. Several oral medications like retinoids, dapsone, ciclosporin and methotrexate have been tried with limited results. Study suggest Calcipotriol cream may be useful in some cases.

In some severe cases Carbon dioxide laser ablation, pulsed dye lasers and dermabrasion have also been tried with some good results. Few research showed that Photodynamic therapy has no success in the treatment of Hailey-Hailey disease. Radiotherapy has also been tried for localized lesions with limited success in few patients. If the skin is severely affected, skin can be removed with surgery followed by skin grafting.

If you have any questions regarding Hailey-Hailey disease ask us at our Dermatalk Skin Care Advice Forum.