Hailey-Hailey Disease

Key Points
Rare, chronic blistering skin condition
Caused by genetic defect, though rare cases have been reported with no family history of the condition
Can be worsened by friction, heat and sweating
Initially consists of a rash in the folds of the skin, accompanied by pain, which clears in the center and becomes ring-shaped as it enlarges

Hailey-Hailey disease is a rare, chronic skin condition which is generally hereditary. It typically forms in areas of the body with skin folds, most commonly the groin, armpits, buttocks, and breasts. The lesions, which initially consist of a painful, blistered rash, may resolve and reappear. Secondary infection of the lesions is common, which can result in a distinct odor coming from the affected area. Where lesions have been present for awhile, the affected area may become thickened, macerated, and form extremely painful cracks in the skin.

While Hailey-Hailey disease may appear at any age, it typically presents in early to mid adult life. It is hereditary, and caused by a gene defect which causes skin cells to stop adhering to each other. In very rare cases, the condition has been seen to appear in persons with no family history of the disease.

Differential Diagnosis (Other conditions with similar appearance)
Erythrasma
Pemphigus
Extramammary Paget Disease
Intertrigo
Pemphigoid gestationis
Pyoderma vegetans
Tinea Cruris

Diagnosis
Key Points
Diagnosis based on symptoms in conjunction with family history
Skin biopsy may be necessary to confirm diagnosis and rule out similar appearing conditions

Diagnosis is based on the presentation of symptoms in conjunction with family history with or without a biopsy showing compatible findings. As it is easily mistaken for other skin conditions, a biopsy may be necessary to confirm diagnosis and to prevent misdiagnosis.

Treatment
No cure exists for the condition
Avoiding things which may trigger the condition can assist in reducing symptoms and flare-ups
Both OTC and prescription treatments can also assist in reducing symptoms
More aggressive treatments may be pursued in severe cases

Generally, Hailey-Hailey disease is harmless outside of the pain and potential for infection. There is no cure, and affected persons will generally see long term exacerbations and remission. Serious flare-ups can occur if a person is infected with the herpes virus, requiring prompt treatment. Avoiding common triggers, such as Sunburn, friction, and sweating can assist in controlling symptoms, as well as wearing absorbent pads in the underwear, can help in preventing outbreaks. Cortisone creams can assist in relieving symptoms and preventing flare-ups, often combined with topical or oral antibiotics for secondary infection.

In persons who require further treatment, the following therapies have been shown to be sometimes effective: Calcipotriol cream, oral antivirals (in the case of recurrent herpes virus infections), botulinum toxin (reduces sweating), phototherapy, and surgical excision. In cases where surgical excision is performed, accompanying skin grafts may be done to reconstruct the affected area.