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Case Reports
. 2000 Sep;143(3):618-20.
doi: 10.1111/j.1365-2133.2000.03721.x.

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

F H Dilek  1 , N Akpolat, A Metin, S Ugras
Affiliations
Case Reports

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

F H Dilek et al. Br J Dermatol. 2000 Sep.

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.

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