Suguna B.V, Hemalata MAHANTAPPA, Aparna MURALİDHAR, Sadaf AHMAD

Kseroderma Pigmentosum ile birlikte görülen Skuamöz Hücre Karsinomu ve Bazal Hücre Karsinomu: Nadir bir vaka sunumu

Kseroderma Pigmentosum, ultraviyole ışın kaynaklı piramidin kalıntılarının çapraz bağlanmasının tamirinde rol oynayan nükleotid eksizyon tamiri aşamasındaki defekt ile karakterize kalıtsal bir DNA onarım bozukluğudur. Etkilenen bireyler normalden çok daha erken yaşta mukokutanöz kanser gelişme riski altındadır. Bu genodermatoza, tüm ırklarda ve her iki cinsiyette rastlamak mümkündür. Xeroderma Pigmentosum’un Hindistan'da görülme sıklığı hala bilinmemektedir. Bu çalışmada dokuz yaşında erkek hasta birlikte skuamöz hücreli karsinom ve yüz bazal hücreli karsinomu olan bir olguyu sunduk.

Rare occurrence of coexistent squamous cell carcinoma and basal cell carcinoma in a case of Xeroderma Pigmentosum

Xeroderma Pigmentosum is an inherited disorder of DNA repair characterised by defective nucleotide excisionrepair, which is involved in repairing ultraviolet rays induced cross linking of pyramidine residues. Affectedindividuals are at increased risk of development of mucocutaneous cancers at a much earlier age than normal.This genodermatosis affects both sexes and all races. The incidence in India is still unknown. We report one suchcase of a nine year old boy with co-existent squamous cell carcinoma and basal cell carcinoma of the face.

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