Xeroderma pigmentosum (XP) is a rare autosomal recessive disorder due to the defect in the nucleotide repair genes resulting in inability to repair the damaged DNA caused by ultraviolet (UV) rays. Hence people suffering from this disease show extreme sensitivity to sunlight and UV radiation and are characterised by skin lesions mostly in the sun-exposed areas such as head, face and neck.
It affects 1 in 250,000 people and has 1000-fold increased risk of developing cancers in sun-exposed areas. Even the incidence of internal malignancy is 10-20 times higher than in normal individuals. The disease may also be associated with progressive neurological degeneration. Most people require multiple surgeries for removal of the skin, ocular lesions and malignancies. The main anaesthetic concerns are difficult intravenous cannulations, difficult airway (bag-mask ventilation and intubation), genotoxic and progressive neurological deterioration of the patients with the use of volatile anaesthetic agents, increased sensitivity to opioids, benzodiazepines and muscle relaxants, and difficult extubation due to epiglottis subsidence.