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Xeroderma pigmentosum (XP) is a rare autosomalrecessiye disorder that occurs because of inactivation of the xeroderma pigmentosum protein, which is an important DNA damage recognition protein involved in DNA nucleotide excision repair (NER). This defect, which prevents removal of a wide array of direct and indirect DNA /esions, is assocrafe d with a decrease in catalase activity. This photosensifive disarder resulfs in multipte face, neck and head basal cell carcinomas (BCCs), squamous cell carcinomas (SCCsJ and melanomas which is characterized by cellular hypersensitivity to ultraviolet radiation, development of cancers at an early age, severe actinic cancer, and photophobia. As a common sfressor of skin, uttraviotet-B (UVB) induces a biphasic HIF-Iq variation through ROS generation in keratinocytes. We reported a case of an 18-year-old-male with XP presented with BCC on the teft cheek. Thc patient had hypo- and hyperpigmented macules since early childhood, throughout the body, more on sun exposed areas. Abstrak Xeroderma pigmentosum (XP) adalah kelainan autosomal resesif yang jarang terjadi disebabkan oleh inaktivasi protein xeroderma pigmentosum yang merupakan protein penting pengenal kerusakan DNA yang terlibat dalam nucleotide excision repair (NER) DNA. Defek ini mencegah penghapusan beragam lesi DNA langsung dan tidak langsung, terkait dengan penurunan aktivitas katalase. Kelainan fotosensitivitas ini menyebabkan terjadinya basal karsinoma sel (BCC), karsinoma sel skuamosa (SCC) dan melanoma pada wajah, leher dan kepala yang ditandaidengan hipersensitivitas seluler radiasi ultraviolet, perkembangan kanker pada usia dini, kanker actinic yang berat, dan fot'ofobia. Ultraviolet-B (UVB) merupakan stressor pada kuit yang menginduksi variasi HIF-1q bifasik melalui generasi ROS dalam keratinosit. Kami melaporkan kasus XP dengan BCC di pipi kiri pada laki-laki berusia 18 tahun. Pasien memiliki makula hipo- dan hiperpigmentasisejak masa kanak-kanak awal, seluruh tubuh, lebih pada daerah yang terpapar sinar matahari.

BASAL CELL CARCINOMA IN XERODERMA PIGMENTOSUM IN AN 18.YEAR.OLD MALE