Late-onset adenosine deaminase deficiency presenting with Heck's disease.

Journal Article (Journal Article)

Focal epithelial hyperplasia, also known as Heck's disease, is a rare but distinctive entity of viral etiology with characteristic clinical and histopathological features. It is a benign, asymptomatic disease of the oral mucosa caused by human papilloma viruses (HPV). Previous studies postulated an association between these lesions and immunodeficiency. Genetic deficiency of adenosine deaminase (ADA) results in varying degrees of immunodeficiency, including neonatal onset severe combined immunodeficiency (ADA-SCID), and milder, later onset immunodeficiency. We report a 12-year-old girl with the late onset-ADA deficiency presenting with Heck's disease. Our case report should draw attention to the possibility of immunodeficiency in patients with HPV-induced focal epithelial hyperplasia.

Full Text

Duke Authors

Cited Authors

  • Artac, H; Göktürk, B; Bozdemir, SE; Toy, H; van der Burg, M; Santisteban, I; Hershfield, M; Reisli, I

Published Date

  • August 2010

Published In

Volume / Issue

  • 169 / 8

Start / End Page

  • 1033 - 1036

PubMed ID

  • 20039061

Electronic International Standard Serial Number (EISSN)

  • 1432-1076

Digital Object Identifier (DOI)

  • 10.1007/s00431-009-1131-9


  • eng

Conference Location

  • Germany