Hailey-Hailey Disease Successfully Treated with Low-Dose Naltrexone

Authors

John Moesch, HCA HealthcareFollow
Richard Miller, HCA HealthcareFollow

Files

Division

West Florida

Hospital

Largo Medical Center

Specialty

Dermatology

Document Type

Poster

Publication Date

5-2020

Keywords

Hailey Hailey Disase, Blistering disease, Naltrexone, Genodermatoses

Disciplines

Dermatology | Heterocyclic Compounds | Skin and Connective Tissue Diseases

Abstract

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).

Recommended Citation

Moesch, John and Miller, Richard, "Hailey-Hailey Disease Successfully Treated with Low-Dose Naltrexone" (2020). West Florida Division GME Research Day 2020. 12.
https://scholarlycommons.hcahealthcare.com/westflorida2020/12