Hailey-Hailey Disease Successfully Treated with Low-Dose Naltrexone

John Moesch; Richard Miller

Hailey-Hailey Disease Successfully Treated with Low-Dose Naltrexone

John Moesch
, Richard Miller

HCA Florida Largo Hospital

HCA Healthcare

Research output: Contribution to journal › Article › peer-review

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).

Original language	American English
Journal	West Florida Division GME Research Day 2020
State	Published - May 1 2020

Keywords

Hailey Hailey Disase
Blistering disease
Naltrexone
Genodermatoses

Disciplines

Dermatology
Heterocyclic Compounds
Skin and Connective Tissue Diseases

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Hailey-Hailey Disease Successfully Treated with Low-Dose NaltrexoFinal published version, 676 KBLicense: Unspecified

Cite this

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title = "Hailey-Hailey Disease Successfully Treated with Low-Dose Naltrexone",

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).",

keywords = "Hailey Hailey Disase, Blistering disease, Naltrexone, Genodermatoses",

author = "John Moesch and Richard Miller",

year = "2020",

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language = "American English",

journal = "West Florida Division GME Research Day 2020",

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T1 - Hailey-Hailey Disease Successfully Treated with Low-Dose Naltrexone

AU - Moesch, John

AU - Miller, Richard

PY - 2020/5/1

Y1 - 2020/5/1

N2 - 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).

AB - 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).

KW - Hailey Hailey Disase

KW - Blistering disease

KW - Naltrexone

KW - Genodermatoses

UR - https://scholarlycommons.hcahealthcare.com/westflorida2020/12

M3 - Article

JO - West Florida Division GME Research Day 2020

JF - West Florida Division GME Research Day 2020

ER -

Hailey-Hailey Disease Successfully Treated with Low-Dose Naltrexone

Abstract

Keywords

Disciplines

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