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 is a rare acquired disorder characterized by diffuse pigmented macules of the oral mucosa. Other mucosal and cutaneous surfaces may be affected, and nail involvement is frequent. A case of Laugier-Hunziker syndrome in a Greek female with multiple buccal and labial mucosal lesions and unusual conjunctival involvement is presented here. In addition, a thorough review of the English language literature of this rather under-recognized syndrome is included and the main differential diagnostic possibilities are discussed.

Laugier-Hunziker syndrome is an acquired macular hyperpigmentation of the oral and genital mucosa, often associated with longitudinal melanonychia. We report a case of longitudinal melanonychia on fingernails and toenails, hutchinson's signal and lenticular pigmentation of her lips and oral mucosa. Histopathological analysis is typical and had a broad differential diagnosis. This is a rare and benign entity, which should be included in the differential diagnosis of mucocutaneous hyperpigmentation.