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Updated: Jun 5, 2026
12:23
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 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.
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.
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