The basal cell nevus syndrome is a rare genetically transmitted disease. It is associated with multiple other organ system abnormalities, the most common of which are multiple dental cysts and other skeletal and skin anomalies. The skin tumors are smooth, rounded papules, flesh colored or pigmented, of varying size, and may appear quite innocent until ulceration occurs. Histologically the lesions are characteristic of basal cell carcinoma from the earliest stages. The patient's family had a history of this syndrome, and he had skeletal and skin anomalies and almost complete destruction of his right upper lid. Treatment consisted of excision and a Cutler-Beard reconstruction. Ophthalmologists should be acquainted with the entity since the first site of involvement frequently is the eyelid.
Markovits AS, Quickert MH. Basal Cell Nevus. Arch Ophthalmol. 1972;88(4):397-399. doi:10.1001/archopht.1972.01000030399009