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Generalized familial benign chronic pemphigus (Hailey-Hailey disease) treated successfully with low-dose naltrexone

Generalized familial benign chronic pemphigus (Hailey-Hailey disease) treated successfully with low-dose naltrexone
Publication Type
Journal Article
Research Type
Reported as
Case Report/Series/Restrospective Study
August 01, 2018
Natalie Kollman and Jonathan Blass
Case Western Reserve University School of Medicine

Hailey-Hailey disease, otherwise known as familial benign chronic pemphigus, is a genetic intraepidermal blistering disorder. In nongeneralized HHD, there have been a few published case reports of HHD treated successfully with low-dose naltrexone (LDN). The exact mechanism by which naltrexone works for HHD is unclear.

The phenotypic expression of HHD can be quite variable. Although at times minimal, in rare cases, this disorder cannot only become generalized but result in systemic sequelae due to fluid and protein loss, with lesions providing an ideal media for bacterial colonization and infection. The use of antibiotics to cover significant pathogens or colonization should be used in combination with other primary treatment modalities. It is unclear how much the antibiotics contributed to resolution of our patient's disease initially; however, the patient clearly showed improvement with only use of naltrexone during weeks 5 through 8. Furthermore, the patient has maintained clearance of his disease using LDN at variable doses and has not used any additional antibiotics. There is a clear correlation between the lowering of the dose and flare of his disease. Because the patient has had no significant side effects from the medication, it has been continued per patient preference.

Until this point, treatments have only modified the disease, as they have not clearly targeted the pathophysiologic components. Despite the uncertainty of mechanism of action, LDN in this patient as well as a few other documented case reports of HHD treated with LDN, led to dramatic clinical improvement, with minimal side effects. Overall, this intriguing topic needs future investigation, as there are limited published data. Based on recent literature and experience with LDN, it would appear to be the treatment of choice for HHD when more conventional treatment modalities fail. Moreover, its use provides a powerful addition to the therapeutic armamentarium for generalized HHD.