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.