Pruritus affects a large proportion of patients with CTCL and is significantly more severe in late- than in early-stage disease and in SS than in MF. Little information exists on the full range of the symptom burden on the patient. This aspect of patient care requires further exploration.

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amilial benign pemphigus, or Hailey-Hailey disease (HHD), is a rare autosomal dominant genetic dermatosis characterized by chronic, recurrent vesicles, erosions, and maceration in flexural areas. It results from mutations in the ATP2C1 gene (UniProt P98194) that lead to defective calcium homeostasis in the Golgi apparatus and ultimately loss of adhesion between keratinocytes. 1 Hailey-Hailey disease is often challenging to treat. Herein, we present a novel treatment option for HHD. MethodsPatients with biopsy-proven recalcitrant HHD were evaluated in the outpatient dermatology clinic at the Cleveland Clinic and treated with low-dose naltrexone hydrochloride (1.5-3.0 mg/d).Because reported dosages in the literature for low-dose naltrexone hydrochloride range from 1.5 to 4.5 mg per day, 1,2 patients were started on 1.5 mg/d and titrated monthly as needed; concomitant topical and systemic medications were ceased. The same physician (one of us, A.P.F.) administered all treatments to all patients and assessed response. Patients were followed up every 2 to 3 months. Clinical response, adverse effects, and subjective quality of life were monitored throughout the treatment. The study dates were January 2016 to January 2017. Because no standardized rating scale exists for HHD to date, response was judged and quantified by taking into account healing of areas of cutaneous breakdown, improvement in erythema, and decrease in patients' reported symptoms or pain and discomfort. No laboratory monitoring was necessary.The department of dermatology determined that institutional review board approval was not necessary for the 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 PARTICIPANTSIn 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 MEASURESObjective 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. RESULTSThe 3 patients included a woman in her 40s and 2 men in their 60s. Each patient exhibited at least an 80% improveme...

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