Abstract
Xeroderma pigmentosum (XP) is a rare, autosomal recessive disease involving a defect in DNA repair leading to the premature development of numerous aggressive cutaneous malignancies. Although atypical fibroxanthoma (AFX) is a neoplasm typically found in the setting of extensive sun exposure or therapeutic radiation, AFXs are rarely associated with children with XP. We report the case of a 13-year-old Guatemalan girl with the XP type C variant who developed one of the largest AFXs reported on a child's finger.
© 2016 Wiley Periodicals, Inc.
MeSH terms
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Adolescent
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Amputation, Surgical / methods
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Biopsy, Needle
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Female
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Fingers / pathology
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Fingers / surgery*
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Guatemala
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Humans
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Immunohistochemistry
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Precancerous Conditions / pathology*
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Rare Diseases
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Risk Assessment
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Skin Neoplasms / complications
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Skin Neoplasms / pathology*
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Skin Neoplasms / surgery
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Treatment Outcome
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Xanthogranuloma, Juvenile / complications
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Xanthogranuloma, Juvenile / pathology*
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Xanthogranuloma, Juvenile / surgery
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Xeroderma Pigmentosum / complications
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Xeroderma Pigmentosum / pathology*
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Xeroderma Pigmentosum / surgery