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Atypical fibroxanthoma of the skin and the lower lip in xeroderma pigmentosum
Author(s) -
Dilek F.H.,
Akpolat N.,
Metin A.,
Ugras S.
Publication year - 2000
Publication title -
british journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.304
H-Index - 179
eISSN - 1365-2133
pISSN - 0007-0963
DOI - 10.1111/j.1365-2133.2000.03721.x
Subject(s) - atypical fibroxanthoma , xeroderma pigmentosum , dermatology , medicine , pathology , immunohistochemistry , trunk , photodermatosis , biology , dna , dna repair , genetics , ecology
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.