Hailey–Hailey disease

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Hailey–Hailey disease
Lesions of Hailey–Hailey disease
Synonyms	Benign chronic pemphigus
Pronounce
Specialty	Dermatology
Symptoms	Blisters, Erosions, Itching, Pain
Complications	Secondary infection
Onset	Usually in adulthood
Duration	Chronic
Types	N/A
Causes	Genetic mutation in the ATP2C1 gene
Risks	Heat, sweating, friction
Diagnosis	Clinical examination, Skin biopsy
Differential diagnosis	Pemphigus vulgaris, Intertrigo, Candidiasis
Prevention	N/A
Treatment	Topical corticosteroids, Antibiotics, Laser therapy
Medication	Corticosteroids, Antibiotics
Prognosis	N/A
Frequency	Rare
Deaths

Hailey–Hailey disease, also known as familial benign chronic pemphigus, is a rare genetic disorder that affects the skin. It was first described by the Hailey brothers in 1939. The condition is characterized by recurrent blisters and erosions, primarily in areas where the skin folds, such as the armpits, groin, neck, and under the breasts.

Signs and Symptoms

The primary symptoms of Hailey–Hailey disease include:

• Painful blisters and erosions
• Red, inflamed skin
• Itching and burning sensations
• Crusting and oozing lesions
• Secondary bacterial infections

These symptoms often worsen with heat, sweating, friction, and stress. The condition typically begins in adolescence or early adulthood and can vary in severity over time.

Genetics

Hailey–Hailey disease is inherited in an autosomal dominant pattern, meaning only one copy of the altered gene is sufficient to cause the disorder. The disease is caused by mutations in the ATP2C1 gene, which encodes a protein involved in calcium transport within cells. This mutation leads to impaired cell adhesion in the epidermis, resulting in the characteristic blisters and erosions.

Diagnosis

Diagnosis of Hailey–Hailey disease is primarily based on clinical examination and patient history. A skin biopsy may be performed to confirm the diagnosis, revealing characteristic findings such as acantholysis, which is the loss of connections between skin cells.

Treatment

There is no cure for Hailey–Hailey disease, but various treatments can help manage symptoms. These include:

• Topical corticosteroids to reduce inflammation
• Antibiotics to treat secondary infections
• Antifungal creams if a fungal infection is present
• Oral retinoids in severe cases
• Laser therapy to reduce lesions
• Botulinum toxin injections to reduce sweating

Patients are also advised to avoid triggers such as heat, friction, and stress, and to maintain good skin hygiene.

Prognosis

The prognosis for individuals with Hailey–Hailey disease varies. While the condition is chronic and can be debilitating, it is not life-threatening. With appropriate management, many individuals can lead relatively normal lives.

See also

• Genetic disorder
• Skin biopsy
• Autosomal dominant
• ATP2C1
• Corticosteroid
• Retinoid
• Botulinum toxin

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