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Improvement of Hailey-Hailey disease with low-dose naltrexone

Improvement of Hailey-Hailey disease with low-dose naltrexone
Publication Type
Journal Article
Research Type
Reported as
Case Report/Series/Restrospective Study
January 07, 2020
M. Jasans‐Barceló P. Curman L. Hagströmer J.D. Wikstrom D. Sairaf
Södersjukhuset Hospital, Karolinska Institute

Hailey-Hailey disease (HHD), or familial benign pemphigus, is a rare autosomal dominant acantholytic dermatosis characterized by blistering, and erythematous, erosive and macerated plaques located primarily in intertriginous areas. Secondary bacterial and viral infections are common. There is no "gold standard" treatment, although many therapeutic modalities have been proposed.

We present a retrospective study that included eight consecutive patients with biopsy-proven HHD who were evaluated at the Departments of Dermato-Venereology at Södersjukhuset and Karolinska University Hospital (Stockholm, Sweden) and treated with low-dose naltrexone (LDN). All but one patient showed a clinical response to treatment as well as improvement in quality of life. Mean improvement of affected BSA was 77% (p<0.01) and DLQI improved with an average of 18 points (p<0.01) measured at 3 months. 

Based on increasing amount of clinical experience, we suggest that LDN should be considered as an early treatment for HHD, e.g. when there is a failed response to topical therapies. LDN is a cheap and seemingly low-risk alternative that may be effective even in severe forms of the disorder. However, a larger exploratory prospective cohort study is needed in order to further substantiate the effect of LDN on HHD. Due to the rarity of this condition, a multi-centre effort using a standardised treatment and follow-up protocol should be the next step forward.