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Hailey-Hailey Disease Successfully Treated with Low-Dose Naltrexone

Hailey-Hailey Disease Successfully Treated with Low-Dose Naltrexone
Publication Type
Research Type
Reported as
Case Report/Series/Restrospective Study
May 31, 2020
John Moesch, Richard Miller
Largo Medical Center

Hailey-Hailey disease (HHD) is an uncommon autosomal dominant disorder resulting from a mutation in the ATP2C1 gene resulting in dysfunction of the Golgi apparatus calcium-associated ATPase, thus interfering with intercellular calcium signaling.

HHD presents clinically as flaccid blisters and erosions in intertriginous areas, especially the axillae and groin. The major histologic finding is acantholysis throughout the spinous layer of the epidermis, commonly referred to as a “dilapidated brick wall” appearance. The initial lesions and associated symptoms usually develop during the second or third decade of life. Complications of HHD include infections (bacterial, fungal, and viral), and malignant transformation (cutaneous squamous cell carcinoma). A 56-year-old female presented to clinic with a worsening rash located on her upper arms, inguinal region, and lower back. Physical exam revealed erythematous, macerated, crusted plaques located on the upper arm, inguinal region, and lower back. The erosions had a “worm-eaten” pattern. Shave biopsy was performed on lesions revealing widespread acantholysis of the epidermis and minimal dyskeratosis. Over the course of several months, the patient was treated with multiple therapies including high potency topical corticosteroids, oral therapy with doxycycline, and two intramuscular injections of 40 mg/ml triamcinolone acetonide. Due to the progressively worsening and recalcitrant nature of the disease, the patient was started on 3 mg/day of oral naltrexone. After 3 months of treatment on 3 mg daily of naltrexone, the patient had almost complete resolution of active lesions on her upper arms, inguinal region, and lower back. The remaining skin changes represent post-inflammatory hyperpigmentation.