Nevoid basal cell carcinoma syndrome. A review and case report of a patient with unilateral basal cell nevus syndrome.

An exceptionally rare example of nevoid basal cell carcinoma syndrome occurring unilaterally is presented, along with a review of the syndrome. We ruled out the possible association of this case with the linear unilateral basal cell nevus with comedones because of several incongruities, including the absence of epidermal cysts and strialike atrophy and the presence of pigmented basal cell carcinomas. Linear unilateral basal cell nevus with comedones is not a variant of the nevoid basal cell carcinoma syndrome and the tumors follow a benign course. The aggressive, destructive behavior of numerous pigmented basal cell carcinomas that involve the eyelid and medial canthus of the eye and the head and neck area represents important evidence that our patient's condition was not an example of linear unilateral basal cell nevus with comedones.