Abstract

Xeroderma pigmentosa (XP) is a rare autosomal recessive disorder which is characterized by a defect in nucleotide excision repair of DNA following exposure to UV radiation. This leads to hypersensitivity to sunlight causing pigmented skin lesions, photophobia and a 1000-fold increase in risk of developing cutaneous malignancies like basal cell carcinomas, squamous cell carcinomas and melanomas of head and neck. We present an interesting case of a 28year old man with basosquamous cell carcinoma secondary to XP treated successfully with surgical excision and reconstruction with rotational flap technique.

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