Files

Download

Download Full Text (675 KB)

Division

West Florida

Hospital

Largo

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).

Hailey-Hailey Disease Successfully Treated with Low-Dose Naltrexone

Share

COinS
 
 

To view the content in your browser, please download Adobe Reader or, alternately,
you may Download the file to your hard drive.

NOTE: The latest versions of Adobe Reader do not support viewing PDF files within Firefox on Mac OS and if you are using a modern (Intel) Mac, there is no official plugin for viewing PDF files within the browser window.