title: Atypical fibroxanthoma of the skin and the lower lip in xeroderma pigmentosum.
abstract: Xeroderma pigmentosum (XP) is a rare, usually autosomal recessive disorder related to DNA repair defects. Atypical fibroxanthoma (AFX) is a pleomorphic tumour that occurs infrequently on the limbs and trunk in children. We report a child with XP who presented with AFX of the facial skin and the lower lip. The diagnosis of AFX was confirmed using histological and immunohistochemical techniques. We discuss the possibility that ultraviolet-induced damage might be implicated in the pathogenesis of AFX.
authors.affiliation: Department of Pathology, Kocatepe University School of Medicine, Afyon, Turkey. ondilek@aku.edu.tr
authors.forename: F H
authors.initials: FH
authors.lastname: Dilek
authors.forename: N
authors.initials: N
authors.lastname: Akpolat
authors.forename: A
authors.initials: A
authors.lastname: Metin
authors.forename: S
authors.initials: S
authors.lastname: Ugras
mesh_terms: D002294:Carcinoma, Squamous Cell / Q000473:pathology; D002675:Child, Preschool; D003937:Diagnosis, Differential; D018324:Hemangioma, Capillary / Q000473:pathology; D018219:Histiocytoma, Benign Fibrous / Q000473:pathology*; D006801:Humans; D008048:Lip Neoplasms / Q000473:pathology*; D008297:Male; D009378:Neoplasms, Multiple Primary / Q000473:pathology*; D012878:Skin Neoplasms / Q000473:pathology*; D014983:Xeroderma Pigmentosum / Q000473:pathology*