Atypical fibroxanthoma of the skin and the lower lip in xeroderma pigmentosum

Xeroderma pigmentosum (XP) is a rare, usually autosomal recessive disorder related to DNA repair defects. Atypical fibroxanthoma (AFX) is a pleomorphi c tumour that occurs infrequently on the limbs and trunk in children, We re port a child with XP who presented with AFX of the facial skin and the lowe r lip, The diagnosis of AFX was confirmed using histological and immunohist ochemical techniques. We discuss the possibility that ultraviolet-induced d amage might be implicated in the pathogenesis of AFX.