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Related Experiment Video

Updated: Jun 5, 2026

Granulocyte-dependent Autoantibody-induced Skin Blistering

Granulocyte-dependent Autoantibody-induced Skin Blistering

Published on: October 12, 2012

Familial benign chronic pemphigus (Hailey-Hailey disease).

Raegan Hunt¹, Kathryn O'Reilly, Jonathan Ralston

¹Department of Dermatology, New York University, New York, NY, USA.

Dermatology Online Journal

|December 18, 2010

Summary

This summary is machine-generated.

This study details an unusual case of familial benign chronic pemphigus, also known as Hailey-Hailey disease. The patient presented with unique, symmetric lesions on the anterior upper body, differing from typical presentations.

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Area of Science:

Dermatology
Genetics
Autoimmune Diseases

Background:

Familial benign chronic pemphigus (Hailey-Hailey disease) is a rare genetic blistering disorder.
Typically, it affects intertriginous areas like skin folds, neck, and groin.
Lesions often present as erosions and vesicles, with a relapsing-remitting course.

Observation:

An atypical case of Hailey-Hailey disease is presented.
The patient exhibited relapsing, flaccid vesicles and erosions.
Lesions were uniquely localized to the upper chest, anterior neck, and anterior upper arms, without intertriginous involvement.

Findings:

This presentation is unusual due to the symmetric, anterior-only localization of lesions.
The patient lacked prior lesions in commonly affected sites.
Relapses were not segmental, despite asymmetric individual eruptions.

Implications:

This case highlights the significant phenotypic variability of Hailey-Hailey disease.
It expands the spectrum of clinical presentations for this genetic skin disorder.
Understanding atypical presentations is crucial for accurate diagnosis and management.