MULTIPLE CUTANEOUS MALIGNANCIES WITH RECURRENCE AND CERVICAL SECONDARIES IN A PATIENT OF XERODERMA PIGMENTOSUM - A RARE CASE PRESENTATION

Abstract : Xeroderma pigmentosum is an autosomal recessive genetic disorder of DNA repair in which the ability to repair damage caused by ultraviolet light is deficient. The basic defect in Xeroderma pigmentosum is a nucleotide excision repair defect, leading to defective repair of DNA damaged by ultraviolet radiation 2. These patients exhibit enhanced sensitivity to ionizing radiation. The result is development of multiple cutaneous malignancies, especially at the sun exposed areas. Early detection of these malignancies is prudent as they are fast growing, metastasize early and lead to death of the patient. This is a case report of such a patient with multiple malignancies- primary, recurrent and with secondary metastatic deposits in the neck.