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Skin-nontumor / Clinical Dermatology

Blistering disorders

Hailey-Hailey disease


Reviewer: Mowafak Hamodat, MB.CH.B, MSc., FRCPC (see Reviewers page)
Revised: 26 August 2011, last major update July 2011
Copyright: (c) 2002-2011, PathologyOutlines.com, Inc.

General
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● Autosomal dominant disorder of crusts, vesicles and erosions in intertriginous regions

Terminology
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● Also called benign familial pemphigus

Clinical features
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● Lesions are frequently itchy and malodorous
● Superinfection by Candida albicans, herpes virus and Staphylococcus aureus are frequent complications
● Lesions occur secondary to mechanical trauma, stress and UV radiation
● May be complicated by squamous cell carcinoma
● Caused by mutations in ATP2C1 gene which encodes the protein hSPCA1; leads to acantholysis of skin (Nat Genet 2000;24:61)

Treatment
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● Waxes and wanes in intensity; possibly antibiotics or steroids (eMedicine), possibly laser therapy (J Cosmet Laser Ther 2011 Jun 21 [Epub ahead of print])

Clinical images
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Various images

Micro description
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● Prominent suprabasal acantholysis, also acantholysis at all levels of epidermis, with upper keratinocytes held only loosely together
● Acantholytic cells may retain some connections and resemble dilapidated bricks
● Adnexal epithelium is usually spared
● Occasionally dyskeratotic cells are seen resembling the corps ronds and grains of Darierís disease
● No/rare inflammation

Micro images
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Suprabasal acantholysis

End of Skin-nontumor / Clinical Dermatology > Blistering disorders > Hailey-Hailey disease


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