Abstract

Xeroderma pigmentosum (XP) is a rare genodermatosis, with a defect affecting recovery of ultraviolet-induced damages and characterized by a high rate of malignancies of the exposed skin areas. Inheritance is autosomal recessive and consanguinity of parents is common. We report two siblings with a history of consanguinity having XP with rare orbital malignancies. The first case had orbital amelanotic melanoma and the second case had sebaceous gland carcinoma (SGC) with orbital extension. In this case series, we have discussed and reviewed orbital malignancies in XP with respect to this report and other cases in the literature. Although early detection and treatment of these malignancies will reduce morbidity and mortality, genetic counselling remains the most important protective measures for XP.