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Low-Dose Naltrexone Treatment of Familial Benign Pemphigus (Hailey-Hailey Disease)

Title
Low-Dose Naltrexone Treatment of Familial Benign Pemphigus (Hailey-Hailey Disease)
Publication Type
Journal Article
Research Type
Human
Reported as
Case Report/Series/Restrospective Study
Date
August 02, 2017
Authors
Omer Ibrahim; Sara R. Hogan; Alok Vij; Anthony P. Fernandez
Institution
Cleveland Clinic Foundation
Link
Abstract

IMPORTANCE Familial benign pemphigus, or Hailey-Hailey disease (HHD), is a rare and debilitating genetic dermatosis characterized by chronic, recurrent vesicles, erosions, and maceration in flexural areas. Despite the reported therapeutic modalities, such as topical and systemic corticosteroids, systemic immunomodulators, topical and systemic retinoids, and laser, HHD can still be markedly difficult to control.

OBJECTIVE To assess low-dose naltrexone hydrochloride in the treatment of recalcitrant HHD.

DESIGN, SETTING, AND PARTICIPANTS In this case series, 3 patients with biopsy-proven recalcitrant HHD were evaluated in the outpatient dermatology clinic at the Cleveland Clinic. Each patient was treated with low-dose naltrexone hydrochloride at a dosage of 1.5 to 3.0 mg per day. No laboratory monitoring was necessary. Clinical response (healing of erosions, improvement in erythema, and alleviation of pain), adverse effects, and subjective quality of life were monitored throughout the treatment. The study dates were January 2016 to January 2017.

MAIN OUTCOMES AND MEASURES Objective clinical response as assessed by the treating dermatologist, subjective quality of life as reported by the patient, and recorded adverse effects were monitored throughout the treatment at intervals of 2 to 3 months.

RESULTS The 3 patients included a woman in her 40s and 2 men in their 60s. Each patient exhibited at least an 80% improvement in extent of disease, with one patient demonstrating 90% clearance. All 3 patients had substantial improvement in quality of life, with one patient reporting improvement in his depression. No adverse effects were recorded.

CONCLUSIONS AND RELEVANCE Low-dose naltrexone may represent a low-cost and low-risk alternative or adjunct in the treatment of HHD.