Dermatofibrosarcoma protuberans in a pediatric patient with ataxia telangiectasia syndrome

Pediatr Dermatol. 2019 May;36(3):400-401. doi: 10.1111/pde.13779. Epub 2019 Mar 10.

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.

Publication types

  • Case Reports

MeSH terms

  • Ataxia Telangiectasia / complications*
  • Child
  • Dermatofibrosarcoma / diagnosis*
  • Dermatofibrosarcoma / etiology*
  • Dermatofibrosarcoma / surgery
  • Female
  • Humans
  • Skin Neoplasms / diagnosis*
  • Skin Neoplasms / etiology*
  • Skin Neoplasms / surgery