Introduction
Xeroderma pigmentosum (XP) is a rare genodermatosis characterized by defective DNA repair, leading to hypersensitivity to ultraviolet (UV) sunlight, resulting in symptoms involving eyes, skin and nervous system. It also predisposes to various cutaneous and non-cutaneous malignancies(1).
Polymorphism in the XP gene is also responsible for individual variations in susceptibility to chemotherapeutic agents(2). Although this theoretically puts xeroderma pigmentosum patients at increased susceptibility to DNA damages by anticancer drugs, literature describing the sensitivity to individual drugs is limited, owing to the rarity of XP itself. In this article, we report a case of cutaneous squamous cell carcinoma in a toddler with XP who had severe toxicity to chemotherapy. We also review the available literature for the same.