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Dermatofibrosarcoma protuberans in a pediatric patient with ataxia telangiectasia syndrome
Author(s) -
Duffy Robert,
Liaqat Maryam,
Lawrence Naomi,
Manders Steven
Publication year - 2019
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1111/pde.13779
Subject(s) - ataxia telangiectasia , dermatofibrosarcoma protuberans , medicine , malignancy , dermatology , telangiectasia , mutation , gene , pathology , genetics , dna , dna damage , biology
Ataxia telangiectasia ( AT ) is a rare autosomal recessive neurodegenerative disorder caused by a mutation in the ATM gene. An impaired immune response due to the gene mutation leads to an increased risk of infection and malignancy. We present a rare case of dermatofibrosarcoma protuberans arising in a patient with AT .
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