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GTR Home > Conditions/Phenotypes > Familial benign pemphigus

Familial benign pemphigus

Synonyms
Benign Chronic Pemphigus; Hailey Hailey disease
Modes of inheritance
Autosomal dominant inheritance (Orphanet)

Summary

Hailey-Hailey disease (HHD), also known as benign chronic pemphigus, is a rare autosomal dominant cutaneous disorder that usually becomes manifest in the third or fourth decade of life with erythema, vesicles, and erosions involving the body folds, particularly the groin and axillary regions. Other sites of the body, such as the neck, perianal, and submammary regions, may likewise be affected (summary by Poblete-Gutierrez et al., 2004). This disorder was first described by the dermatologist brothers Hailey and Hailey in 1939 (see Michel, 1982). [from OMIM]

Available tests

19 tests are in the database for this condition.

Check Related conditions for additional relevant tests.

Clinical tests (19 available)

Molecular Genetics Tests

Genes See tests for all associated and related genes

  • Also known as: ATP2C1A, BCPM, HHD, PMR1, SPCA1, hSPCA1, ATP2C1
    Summary: ATPase secretory pathway Ca2+ transporting 1

Clinical features

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Imported from Human Phenotype Ontology (HPO)

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