Lentigo maligna in a woman with oculocutaneous albinism.

A 54-year-old black woman with oculocutaneous albinism was found to have a lentigo maligna and, subsequently, a pigmented compound nevus on non-sun-exposed areas of her body. The diagnosis for these lesions was confirmed by the results of histopathologic study. To our knowledge, this is the first such case reported in the literature. This patient reemphasizes the fact that melanoma development is dependent on the presence of melanocytes, but not necessarily on melanogenesis. We propose that the rarity of melanomas in these patients may be caused by the protective effect of unknown systemic factors.