Dermatofibrosarcoma protuberans in a pediatric patient with ataxia telangiectasia syndrome.

Duffy R¹, Liaqat M², Lawrence N², Manders S¹.

Author information

1: Cooper Medical School of Rowan University, Camden, NJ.
2: Division of Dermatology, Cooper University Hospital, Camden, NJ.

Abstract

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

PMID:: 30854690
DOI:: 10.1111/pde.13779