LAUGIER-HUNZIKER SYNDROME

By Gary M. White, MD

Laugier– Hunziker syndrome.  Lesions of the tongue


Laugier-Hunziker syndrome (LHS) is a rare, benign, idiopathic condition characterized by acquired macular pigmentation of various sites, but primarily the lips and buccal mucosa, often accompanied with melanonychia. Key to the diagnosis is the lack of systemic associations, e.g., polyposis. Often patients have a forme fruste of the disease, i.e. pigmentation at only one or two sites.

Clinical

Patients with Laugier–-Hunziker syndrome develop pigmented macules of the lips, tongue, oral mucosa (e.g., palate), and/or the nail apparatus (e.g., longitudinal melanonychia) in the absence of systemic disease. Other sites such as the fingertips, palms, feet, esophagus, and vulva have been reported to be involved. The lesions may be brown–, black, or bluish-black.  

Only oral pigmentation may occur, or both oral and nail pigmentation. Pale-skinned people are affected almost exclusively. Histology shows increased pigmentation in the basal layer, along with pigmentary incontinence.

Treatment

No treatment is needed but the laser may remove cosmetically unsightly lesions.

Additional Pictures

Laugier– Hunziker syndrome.  Pigmentation of the lips and tongue Laugier– Hunziker syndrome nails

Laugier– Hunziker syndrome.  Pigmentation of the lips Laugier– Hunziker syndrome.

Laugier– Hunziker syndrome.

References

Pigmentation on the nails, lips, and palate. CMAJ. 2011 Sep 6; 183(12): 1402

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