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.
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.