Laugier-Hunziker syndrome with familial distribution

Laugier-Hunziker syndrome (LHS) is an acquired melanosis of lips and/or oral mucosa, in half the cases associated with melanonychia striata and, rarely, with hyperpigmentation of other mucocutaneous districts. LHS with familial distribution has rarely been reported in the literature; notwithstanding, at present it is considered a not uncommon condition. Histology is characterized by increased melanization of the lower layers of epidermis with no expansion of the melanocyte population. 3 relatives (mother and 2 daughters) with LHS of the oral cavity are reported. Differential diagnosis with other causes of oral pigmentation is discussed. Due to the familial distribution, Peutz-Jeghers syndrome must be differentiated for the absence of systemic disturbances and the normal number of melanocytes in the pigmented macules.