By Gary M. White, MD
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 with its associated polyposis.
- The pigmentation may appear identical to that of 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.
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.
No treatment is needed but the laser may remove cosmetically unsightly lesions.
Pigmentation on the nails, lips, and palate. CMAJ. 2011 Sep 6; 183(12): 1402
Homepage | FAQs | Use of Images | Contact Dr. White
It is not the intention of RegionalDerm.com to provide specific medical advice, diagnosis or treatment. RegionalDerm.com only intends to provide users with information regarding various medical conditions for educational purposes and will not provide specific medical advice. Information on RegionalDerm.com is not intended as a substitute for seeking medical treatment and you should always seek the advice of a qualified healthcare provider for diagnosis and for answers to your individual questions. Information contained on RegionalDerm.com should never cause you to disregard professional medical advice or delay seeking treatment. If you live in the United States and believe you are having a medical emergency call 911 immediately.