The congenital lingual melanotic macule.

BACKGROUND Circumscribed oral melanotic macules in adults are a well-defined entity. Congenital oral melanotic macules, however, are rare and not well characterized. We report 5 cases of congenital lingual melanotic lesions with consistent clinical and histologic features. OBSERVATIONS Congenital lingual melanotic macules presented in all cases at birth, followed by proportional growth. Clinical findings were well-circumscribed, brown, single or multiple macules on the tongue, 2 to 5 mm in size, without history of bleeding, ulceration, or trauma. Family history was negative for similar pigmented lesions. A literature review showed only 2 previous clinical case reports with similar features. Histopathologic examination showed increased melanin pigmentation in the basal epidermal layer with varying degrees of overlying hyperkeratosis and subepidermal pigment-laden macrophages. No appreciable increase in melanocyte number, junctional nests of melanocytes, or cell atypia was noted. Two retrospective histopathology reviews mention 4 corresponding cases but report no clinical data. CONCLUSION The congenital lingual melanotic macule represents a clinically distinct, benign, pigmented oral melanotic lesion, and may be more common than the literature suggests.