Labial and oral melanotic macules are commonly encountered in a broad range of conditions ranging from physiologic pigmentation to a sign of an underlying life-threatening disease. Although Laugier-Hunziker syndrome (LHS) shares some features of labial and oral pigmentation with a variety of conditions, it is a benign and acquired condition, frequently associated with longitudinal melanonychia. Herein, the demographic, clinical, dermoscopic, and pathological aspects of LHS were reviewed comprehensively. The important differential diagnoses of mucocutaneous and nail pigmentation are provided. An accurate diagnosis is crucial to design a reasonable medical strategy, including management options, malignant transformation surveillance, and psychological support. It is important that clinicians conduct long-term follow-up and surveillance due to the potential risks of malignant transformation and local severe complications in some conditions.

Laugier-Hunziker syndrome (LHS), a rare, acquired pigmentary disorder of the lips, oral mucosa, and fingers, is known to be an entirely benign disease with no systemic manifestations. In the past, the pigmentation has been treated efficiently in a few patients with the Q-switched neodymium: yttrium-aluminum-garnet (Nd:YAG) laser and the Q-switched alexandrite laser (QSAL). In order to evaluate the efficacy and safety of QSAL on Chinese patients of LHS, we treated 22 patients with QSAL in the past 5 years. Treatments were delivered on a bimonthly or trimonthly basis until the abnormal pigmentation totally disappeared. Patients were evaluated at each visit for evidence of dyspigmentation, scarring, or other untoward effects from the laser treatment. Our 22 subjects consisted of 18 females and 4 males with a mean age of 42.4 years. After only one session of laser treatment, the clearing on the lips was as follow: 18 (81.8%) excellent, 2 (9.1%) good, 1 (4.5%) fair and 1 (4.5%) poor. Eighteen patients (81.8%) with LHS, who had achieved excellent clearing after only one session of laser treatment, did not receive further treatment. Among the left four patients, three patients (13.6%) achieved complete results after three laser treatments. Only one patient required six sessions to achieve complete clearance. No scarring was noted after any of the treatments. The appearance of pigmentation on mucous membranes in a middle-aged patient without a significant family history for skin disorders should prompt consideration for the possible diagnosis of LHS. Our study has also demonstrated QSAL to be highly effective and safe in the treatment of LHS.

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.

Laugier-Hunziker (LH) syndrome is a rare benign condition in which hyperpigmentation of the lips and buccal mucosa occurs with no systemic associations.

We report the response to treatment with the Q-switched alexandrite laser (QSAL) because there are few reports on therapy for LH syndrome.

The QSAL was used for pigmentation of the lips in a 63-year-old woman with LH syndrome. Laser irradiation was done at 5.0 J/cm2 with a 3 mm spot size.

There was 100% clearance of pigmentation of the lips with a single laser treatment, and recurrence was not observed after 6 months.

The QSAL is very effective for pigmentation owing to LH syndrome.