Hailey-Hailey disease
Findings
No curated finding names Hailey-Hailey disease yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Benign chronic familial pemphigus of Hailey-Hailey is characterized by rhagades mostly located in the armpits, inguinal and perineal folds (scrotum, vulva).
Definition from the Mondo Disease Ontology (MONDO:0008218), read 2026-09-29. CC BY 4.0.
Features
5 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- AcantholysisHPOHP:0100792
- Very frequent (80% to 99% of cases)
- ErythemaHPOHP:0010783
- Very frequent (80% to 99% of cases)
- HyperkeratosisHPOHP:0000962
- Very frequent (80% to 99% of cases)
- Skin erosionHPOHP:0200041
- Very frequent (80% to 99% of cases)
- Skin vesicleHPOHP:0200037
- Very frequent (80% to 99% of cases)
Genes
1 gene
Germline variants in the gene encoding each protein, as classified by GenCC submitters, each in their own words. Limited, disputed and refuted submissions are listed too.
- ATP2C1HGNC:13211
- Definitive · Ambry Genetics · Autosomal dominant · 2024
- Definitive · G2P · Autosomal dominant · 2018
- Strong · Genomics England PanelApp · Autosomal dominant · 2020
- Strong · Labcorp Genetics (formerly Invitae) · Autosomal dominant · 2021
- Strong · PanelApp Australia · Autosomal dominant · 2025
- Supportive · Orphanet · Autosomal dominant · 2021
Where it sits
Other names
3 names
Resolves to: Hailey-Hailey disease
- Also called
- benign chronic familial pemphigus of Hailey-Haileybenign chronic pemphiguspemphigus, benign familial