HAILEY-HAILEY DISEASE

By Gary M. White, MD

Hailey-Hailey Disease


Hailey-Hailey Disease (HHD), AKA benign familial pemphigus, is a rare, autosomal dominant genodermatosis characterized by erosions, vesicles, and bulla predominantly in the flexural areas.

Clinical           

Wet tissue paper erosions or erythematous plaques are found symmetrically in the intertriginous areas where the skin surfaces oppose each other. Most common sites in descending order are groin, axilla, perineum, inframammary region, umbilicus, and retroauricular area.

Treatment   

The defect of Hailey-Hailey disease (HHD) causes the skin to be more fragile. Thus, reducing friction, heat, and sweat is imperative. Topical steroids are anti-inflammatory but because HHD typically involves the flexures, skin atrophy can be a problem. Oral antibiotics (e.g., tetracycline, doxycycline, cephalexin) are usually given to reduce bacterial colonization and infection.

To reduce the surface bacteria, bleach baths, as are used in atopic dermatitis, can be helpful.

Botulinum toxin to reduce sweating has been effective in several reports [Derm Surg 2000;26;371] and can be recommended for recalcitrant disease, especially in the inguinal, inframammary or axillary areas. Treatment must be repeated. Oral glycopyrrolate 1 mg/day was very effective in one patient with refractory HHD [JAMA Derm 2015;151;328]. Higher doses may be need.

Dermabrasion or laser vaporization (CO2 or YAG [AD 1999;135;423]) can induce long-term remission in localized areas. (It appears that the defect of Hailey-Hailey disease is limited to the epidermal keratinocytes and spares the adnexal structures from which reepithelialization occurs.) Dermabrasion Pulsed dye laser can be helpful, but results are variable [JAAD 2015;72;735]. Electron Beam Therapy can induce remission for localized disease [JAAD Case Rep. 2016 Mar; 2(2): 159–161].

Low dose naltrexone (1.5 mg/day and increased if needed to 3.0 mg/day) lead to 80% improvement or more in 3 patients [JAMA Dermatol 2017;153:1015]. No laboratory monitoring was necessary. Full-dose naltrexone therapy is 50-100 mg daily. At this low dose, reported side effects include vivid dreams and headache. A second study in the same journal treated another three patients with 3 mg nightly, titrated to 4.5 mg nightly in 2 patients [JAMA Dermatol 2017;153:1018]. Complete clinical resolution in all 3 patients occurred within 2 months. Lesions flared when stopping low-dose naltrexone and cleared within a few days on rechallenge. Low-dose naltrexone must be compounded because there is no commercial formulation of naltrexone at low dosage. Naltrexone is an opioid receptor antagonist FDA-approved for opioid addiction. The typical dose for opioid addiction is 50-100 mg/day.

Miscellaneous

Additional Pictures

The classic wet-tissue paper morphology.
Hailey-Hailey Disease

HHD of the Axilla

Hailey-Hailey Disease Hailey-Hailey Disease

Hailey-Hailey Disease Hailey-Hailey Disease

Crusted, Annular Lesions on the Trunk

Hailey-Hailey Disease Hailey-Hailey Disease Hailey-Hailey Disease Hailey-Hailey Disease

HHD of the Groin

Hailey-Hailey Disease Hailey-Hailey Disease

Hailey-Hailey Disease Hailey-Hailey Disease

RegionalDerm

Homepage | FAQs | Use of Images | Contact Dr. White


It is not the intention of RegionalDerm.com to provide specific medical advice, diagnosis or treatment. RegionalDerm.com only intends to provide users with information regarding various medical conditions for educational purposes and will not provide specific medical advice. Information on RegionalDerm.com is not intended as a substitute for seeking medical treatment and you should always seek the advice of a qualified healthcare provider for diagnosis and for answers to your individual questions. Information contained on RegionalDerm.com should never cause you to disregard professional medical advice or delay seeking treatment. If you live in the United States and believe you are having a medical emergency call 911 immediately.