Bilateral nevus of Ota in association with nevus flammeus: a case report

Nevus of Ota and nevus flammeus are distinct congenital pigmentary and vascular anomalies, respectively, with different etiologies and clinical implications. While both conditions can present unilaterally, their bilateral coexistence is rare. We report a 39-year-old Filipino female with a medical history of type 2 diabetes mellitus and dyslipidemia who presented to the dermatology clinic with concerns over a newly appearing benign nevus on the sole of her hand. Incidentally, dermatological examination revealed two significant congenital pigmentary lesions: bilateral nevus of Ota and a widespread port-wine stain. The nevus of Ota manifested as well-defined blue-gray macules and patches symmetrically distributed across her face, involving both nasal nostrils and conjunctiva. The nevus flammeus, a congenital vascular malformation, extended over both arms, the upper back, and the chest, presenting as a well-demarcated reddish-purple discoloration. These lesions had been present since birth, remained asymptomatic, and had never been previously evaluated. No systemic involvement was detected, and laboratory investigations were unremarkable. The diagnosis was made clinically. As the patient was not interested in treating the nevi, no treatment was planned. This case highlights the importance of obtaining a detailed family history in all similar cases to aid in understanding the genetic basis of these conditions and their potential coexistence with other congenital dermal anomalies.