All of the following are true about Xeroderma pigmentosum except:
Xeroderma Pigmentosum At increased risk for the development of cancers of the skin paicularly following exposure to the UV light contained in sun rays. UV radiation causes cross-linking of pyrimidine residues, preventing normal DNA replication. Such DNA damage is repaired by the nucleotide excision repair system. Increased risk of both melanocytic and non-melanocytic cancer. Eg : Xeroderma Pigmentosum Big FAX (Inherited disorder of DNA repair) Big: Bloom syndrome F: Fanconi anemia A: Ataxia telangiectasia X: Xeroderma pigmentosum
Skin manifestations appear within first 2 years of life
Increased risk of non melanotic skin cancers
Nucleotide excision repair defect
Autosomal dominant inheritance
JIPMER 2017