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A case of xeroderma pigmentosum complicated with squamous cell carcinoma is presented. A 15 year old male has had a history of a single walnut-sized tumorous growth of 6 months duration on tip of his nose and multiple light to dark brown -pigmented macules on face, ears, neck, upper chest, extensor surface of both forearms and dorsal hands of about 14 years duration, recognizable photosensitivity of the cutaneous lesions and photophobia and lacrimation since early childhood. Xeroderma pigmentosum was suspected under clinical background. Biopsy was performed on the facial pigmented lesions- and on the tumorous mass on the nose, which revealed xeroderma pigmentosum and squamous cell carcinoma, respectively. The tumor was excised totally under local 21% procaine anesthesia, resulting in healing with scar formation and no recurrence was observed during 6 month´s follow up. Dermabrasion of the entire face was done, with complete removal of the xerodermic skin lesions, but they all recurred after about 1 month.
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