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Hailey-Hailey Disease

An inherited disease characterized by recurrent erosive and bullous lesions in flexural areas.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Dermatoloji department. Book Appointment →

What is Hailey-Hailey Disease?

Hailey-Hailey disease (familial benign pemphigus) is a chronic autosomal dominant inherited skin disease in which keratinocyte adhesion is impaired due to mutations in the ATP2C1 gene.

Clinically, recurrent erythematous plaques, small blisters and painful erosions develop in the neck, armpits, groin and perianal area. Heat, sweating, friction and infections trigger flares.

It is not malignant but seriously affects quality of life and treatment requires continuity.

Symptoms

Moist, red plaques in flexural areas
Small blisters and painful erosions
Fissures and cracks
Bad odor and secondary bacterial infection
Itching and burning sensation
Pronounced flares in summer months
Recurrent attacks in the same area

Risk Factors

Family history of Hailey-Hailey disease
ATP2C1 gene mutation
Hot and humid climate
Excessive sweating (hyperhidrosis)
Mechanical friction and tight clothing
Fungal and bacterial infections
Obesity

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • When there are recurrent painful eruptions in flexural areas
  • When lesions become infected and fever develops
  • When widespread flares uncontrolled by treatment occur
  • When daily life is severely affected
  • For genetic counseling if there is a family history

Treatment Methods

01
Topical corticosteroids and calcineurin inhibitors
02
Antibiotic and antifungal treatments (secondary infections)
03
Oral antibiotics (long-term prophylactic)
04
Botulinum toxin injection (for excessive sweating)
05
Laser, photodynamic therapy or surgery in resistant cases
06
Lifestyle measures reducing heat, friction and sweating

Which Department to Visit?

You can visit our Dermatoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Let us help you

You can make an appointment with our specialists or contact us for your concerns.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.