Unraveling the Anesthesia Enigma in Xeroderma Pigmentosum: A Case Report

Xeroderma pigmentosum (XP) is a rare autosomal recessive disorder.It was first described by Kaposi in the 1870s.Its incidence ranges from 1 per 20,000 in Japan to 2.3 per 1 million live births in the United States, 1,2 with more cases in North Africa and the Middle East. 3 However, data about the incidence in India remains limited.It affects all races and both sexes, with a higher incidence in areas with consanguinity. 4 Xeroderma pigmentosum is marked by an extreme sensitivity to ultraviolet (UV) radiation and progressive neurological complications stemming from a deoxyribonucleic acid (DNA) repair defect.It manifests in early childhood, and approximately 60% die before the age of 20 years. 5,6The most common presentations include cutaneous (100%), ophthalmic (75%), and neurological symptoms (25%). 6eroderma pigmentosum may present with sunburn reactions, freckle-like pigmentation, cutaneous and noncutaneous tumors, ocular pathologies (photophobia, conjunctivitis, corneal drying and ulcer,