Publication:

Laugier-Hunziker syndrome with xanthelasma palpebrarum in an adolescent girl: a rare association

Laugier-Hunziker syndrome (LHS) is a rare and benign acquired disorder of mucocutaneous pigmentation characterized by hyperpigmented macules over the lips, buccal mucosa and acral sites with associated nail pigmentation. It usually occurs in middle-aged adults with a female preponderance. The pigmentary lesions respond poorly to therapy and has high chance of recurrence. The syndrome has neither malignant predisposition nor any underlying systemic abnormality. LHS is a diagnosis of exclusion. Clinical correlation is needed to rule out other causes of mucocutaneous hyperpigmentation. It is an important differential diagnosis to Peutz Jeghers syndrome. Herein we report a case of a 16-year-old female presenting with a 10-year history of asymptomatic pigmentation of oral and acral regions with nail pigmentation and was diagnosed with LHS after ruling out other possible differential diagnoses. The patient also had lesions corresponding to xanthalesma palpebrarum in the medial aspect of both the upper eyelids. Both LHS and xanthalesma palpebrarum are very unusual conditions to present in this age.