. This 40-year-old woman complains of new onset dark spots on the tongue, lips and nails. A recent colonoscopy was negative.

LAUGIER-HUNZIKER SYNDROME

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.

• The key is to distinguish LHS from other syndromes with internal associations, e.g., Peutz-Jeghers syndrome. Thus, an upper GI tract barium enema or colonoscopy may be needed to exclude colonic polyposis.
• Onset is in adult life, with women affected more commonly than men.
• Familial cases have been reported. 

Clinical

Patients with LHS 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.

RegionalDerm

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