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.
Keywords: ataxia telangiectasia syndrome; dermatofibrosarcoma protuberans.
© 2019 Wiley Periodicals, Inc.