Ann Dermatol.  2008 Sep;20(3):126-129. 10.5021/ad.2008.20.3.126.

A Case of Laugier-Hunziker Syndrome

• Kim EJ
• Cho SH
• Lee JD

• KMID: 2156377
• DOI: http://doi.org/10.5021/ad.2008.20.3.126

Abstract

Laugier-Hunziker syndrome is a rare idiopathic acquired hyperpigmentation of oral mucosa and lips which is often associated with longitudinal melanonychia. This condition is known to be an entirely benign disease with no systemic manifestations. Herein we report a case of a 54-year-old woman presenting with a 7-year history of asymptomatic oral pigmentation and linear pigmented streaks of several fingernails. Histological examination taken from the mucosal lesion of tongue revealed increased pigmentation at the epithelial basal layer and a few melanophages in the submucosa. The lack of a family history coupled with the absence of any polyps on the endoscopic examinations of upper and lower bowel as well as the clinical features with late onset were all in favor of a diagnosis of Laugier-Hunziker syndrome. The significance of this disease is due to the need to differentiate this disorder from other pigmentary disorders of the oral mucosa, especially Peutz-Jeghers syndrome.

Keyword

Laugier-Hunziker syndrome; Melanonychia; Oral pigmentation

MeSH Terms

Female
Humans
Hyperpigmentation
Lip
Middle Aged
Mouth Mucosa
Nails
Peutz-Jeghers Syndrome
Pigmentation
Polyps
Tongue

Figure

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  Fig. 1 (A) Dark brown to black, confluent, hyperpigmented macules on the lips and intraoral mucosa. (B) Diffuse hyperpigmentation on the lateral surface of tongue. (C) Longitudinal melanonychia of the right index fingernail. Hyponychium also shows hyperpigmentation.

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  Fig. 2 Histological examination from tongue mucosal lesion. (A) Basal layer hyperpigmentation and superficial pigment incontinences (H&E stain, original magnification: ×400). (B) Increased melanin pigments in the basal layer of mucosal epithelium and melanophages in the submucosa (Fontana-Masson, original magnification: ×400). (C) No melanocytic hyperplasia was detectable (Immunohistochemical staining for S-100, original magnification: ×400).

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