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Laugier-Hunziker syndrome
See also in: Oral Mucosal Lesion
Other Resources UpToDate PubMed

Laugier-Hunziker syndrome

See also in: Oral Mucosal Lesion
Contributors: Chase W. Kwon MD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Laugier-Hunziker syndrome (LHS) is a rare, benign condition of macular hyperpigmentation of mucocutaneous surfaces and nails. Although a limited number of familial cases have been reported, no putative genetic defects have been identified, and this is generally thought to be an acquired condition. There are fewer than 200 reported cases in the literature, with the highest prevalence among individuals of Northern European descent. Women are also more frequently affected, with a female to male ratio of 2 to 1. This disorder typically manifests in middle-aged adults as gradual and progressive macular pigmentation of mucocutaneous surfaces, often involving the lips and oral mucosa, as well as the nails.

The etiology of this disorder remains unclear. Histopathological examinations of affected individuals suggest a functional alteration of melanocytes, which appear to be activated to continually synthesize higher amounts of melanin in a chronic fashion. However, the initial stimulus that induces this melanocyte activation is unknown.

Recognizing that this syndrome may mimic other pigmentary disorders, it is important to rule out those with potential for malignant transformation or other clinical implications. As a benign condition itself, the macular lesions of this syndrome do not require therapeutic intervention, although treatment may be pursued for cosmesis.

Codes

ICD10CM:
K13.70 – Unspecified lesions of oral mucosa

SNOMEDCT:
238706002 – Laugier-Hunziker syndrome

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Last Reviewed:02/26/2017
Last Updated:02/26/2017
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Laugier-Hunziker syndrome
See also in: Oral Mucosal Lesion
A medical illustration showing key findings of Laugier-Hunziker syndrome : Hyperpigmented macule, Longitudinal pigmented nail bands, Oral brown macule, Oral gray macule, Oral pigmented macule/patch
Clinical image of Laugier-Hunziker syndrome - imageId=1653517. Click to open in gallery.  caption: 'Longitudinal pigmented bands of a fingernail and grayish macules on the lower lip.'
Longitudinal pigmented bands of a fingernail and grayish macules on the lower lip.
Copyright © 2024 VisualDx®. All rights reserved.