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Dermatol Ther. 2019 Apr 8:e12892. doi: 10.1111/dth.12892. [Epub ahead of print]

Use of low-dose naltrexone in the treatment of severe hailey-hailey disease: one case report.

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Department of Dermatology, Hospital Clínico Universitario de Valladolid, Valladolid, Spain.



Hailey-Hailey disease (HHD) or chronic benign familial pemphigus is an autosomal dominant genodermatosis with complete penetrance characterized by painful vesicles, erosions and macerated intertriginous skin.


We present a 66-year-old woman with a personal 35-year history of pruritic recurrent vesicles and erosions in both axillae and inguinal folds. HHD was confirmed by cutaneous biopsy. Past treatments had failed, including topical corticosteroids, antibiotics and oral doxycycline, minocycline, dapsone and acitretin. Phototherapy and intralesional injection of toxin botulinum A was performed in the axillae. The patient was started on naltrexone 6,25mg nightly. Six weeks later, complete clearing was observed.


At typical doses, naltrexone blocks μ and δ opiod receptors, thereby blocking the union of β -endorphins at those sites. Paradoxically, at low doses, the partial binding to those receptors leads to a homeostatic increase of opioid receptors and an upregulation of endogenous opioids. Low-dose naltrexone may also exert an anti-inflammatory action through its antagonist effect on toll-like receptor 4 found on macrophages.


We consider that low-dose naltrexone is an effective and safe alternative for the HHD, representing an important progress in the management of this disease with limited therapeutic options. This article is protected by copyright. All rights reserved.


Naltrexone; familial chronic benign pemphigus; hailey-hailey


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