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Hailey-Hailey disease
See also in: Anogenital
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Hailey-Hailey disease

See also in: Anogenital
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Contributors: Susan Burgin MD, Belinda Tan MD, PhD, Lowell A. Goldsmith MD, MPH
Other Resources UpToDate PubMed

Synopsis

Hailey-Hailey disease, also known as benign familial pemphigus, is an autosomal dominant disease affecting intertriginous skin, most commonly in the groin, axillae, and lateral neck. The inframammary area and antecubital fossae may be involved less frequently, as may non-intertriginous skin. The primary flaccid vesicles and erosions that develop as a result of loss of keratinocyte cohesiveness may become secondarily infected with bacteria or yeast and may itch mildly. The disease is aggravated by hot and humid weather.

For more information, see OMIM.

Codes

ICD10CM:
Q82.8 – Other specified congenital malformations of skin

SNOMEDCT:
79468000 – Hailey-Hailey disease

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

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Management Pearls

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Therapy

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References

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Last Updated: 03/29/2017
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Hailey-Hailey disease
See also in: Anogenital
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Hailey-Hailey disease : Axilla, Burning skin sensation, Inframammary fold of chest, Intertriginous, Inguinal region, Skin erosions
Clinical image of Hailey-Hailey disease
A well-demarcated, macerated, erythematous plaque with many linear and few tiny round erosions in the axilla.
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