Benign Chronic Pemphigus

Alternative Names

  • Pemphigus, benign Familial
  • Hailey-Hailey Disease
  • HHD
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WHO-ICD-10 version:2010

Congenital malformations, deformations and chromosomal abnormalities

Other congenital malformations

OMIM Number

169600

Mode of Inheritance

Autosomal dominant

Gene Map Locus

3q22.1

Description

Hailey-Hailey disease, 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. [From OMIM]

Epidemiology in the Arab World

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Subject IDCountrySexFamily HistoryParental ConsanguinityHPO TermsVariantZygosityMode of InheritanceReferenceRemarks
169600.1LebanonYes Abnormal blistering of the skin; Skin e...NM_014382.4:c.13C>THeterozygousAutosomal, DominantBtadini et al. 2015
169600.2LebanonYes Abnormal blistering of the skin; Skin ...NM_014382.4:c.361-1G>AHeterozygousAutosomal, DominantBtadini et al. 2015
169600.3LebanonYes Abnormal blistering of the skin; Skin e...NM_014382.4:c.2371_2374delTTGTHeterozygousAutosomal, DominantBtadini et al. 2015
169600.4LebanonYes Abnormal blistering of the skin; Skin e...NM_014382.4:c.13C>THeterozygousAutosomal, DominantBtadini et al. 2015
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