Cutaneous malignancy in albinism.

Albinism is a disorder of hypopigmentation affecting the skin, appendages, and eyes. Ultraviolet light-induced cutaneous tumors are common in patients with albinism due to reduced or absent protection from melanin. Squamous cell carcinoma (SCC) is the number one skin tumor seen in patients who are albinos. Although nonmelanomatous skin cancers are more common in patients with albinism, dysplastic nevus and melanoma present a greater diagnostic challenge in this group because of their hypopigmented appearance. We report 2 cases of cutaneous malignancies in patients who had oculocutaneous albinism (OCA). The first case involves a 45-year-old man with OCA type 2 (OCA2) who developed a large SCC of the neck. The second case involves a 24-year-old man with Hermansky-Pudiak syndrome (HPS) who developed amelanotic melanoma. In both cases, hypopigmentation of the lesions contributed to a delay in diagnosis. We review the clinical, diagnostic, and therapeutic concerns for patients with albinism who have cutaneous malignancies.