158. Sarcoidosis

  1. Alan D. Irvine MD, FRCPI, FRCP3,4,
  2. Peter H. Hoeger MD5,6 and
  3. Albert C. Yan MD, FAAP, FAAD7,8
  1. Julie L. Cantatore-Francis MD1 and
  2. Julie V. Schaffer MD2

Published Online: 24 MAY 2011

DOI: 10.1002/9781444345384.ch158

Harper's Textbook of Pediatric Dermatology, Volume 1, 2, Third Edition

Harper's Textbook of Pediatric Dermatology, Volume 1, 2, Third Edition

How to Cite

Cantatore-Francis, J. L. and Schaffer, J. V. (2011) Sarcoidosis, in Harper's Textbook of Pediatric Dermatology, Volume 1, 2, Third Edition (eds A. D. Irvine, P. H. Hoeger and A. C. Yan), Wiley-Blackwell, Oxford, UK. doi: 10.1002/9781444345384.ch158

Editor Information

  1. 3

    Trinity College, Dublin, Ireland

  2. 4

    Our Lady's Children's Hospital, Dublin, Ireland

  3. 5

    University of Hamburg, Hamburg, Germany

  4. 6

    Catholic Children's Hospital Wilhelmstift, Hamburg, Germany

  5. 7

    University of Pennsylvania School of Medicine, Philadelphia, PA, USA

  6. 8

    The Children's Hospital of Philadelphia, Philadelphia, PA, USA

Author Information

  1. 1

    State University of New York Downstate Medical Center, New York, NY, USA

  2. 2

    Director of Pediatric Dermatology, Department of Dermatology, New York University School of Medicine, New York, NY, USA

Publication History

  1. Published Online: 24 MAY 2011
  2. Published Print: 3 JUN 2011

ISBN Information

Print ISBN: 9781405176958

Online ISBN: 9781444345384



  • Blau syndrome;
  • early-onset sarcoidosis;
  • granulomatous arthritis (familial or pediatric);
  • granulomas;
  • juvenile systemic granulomatosis;
  • preschool sarcoidosis;
  • sarcoidosis


Sarcoidosis is a multisystem disorder characterized by the development of non-caseating granulomas. Two distinct forms of sarcoidosis occur in paediatric patients. School-aged children and adolescents can develop classic sarcoidosis with manifestations similar to the disease in adults, including frequent hilar lymphadenopathy, pulmonary involvement and a wide variety of cutaneous findings. The latter can include both lesions characterized histologically by non-caseating granulomas, most often manifesting as red–brown papules and plaques that favour the face, and reactive lesions such as erythema nodosum. In contrast, infants and preschool-aged children typically present with a triad of arthritis, uveitis and papular granulomatous dermatitis that has been referred to as early-onset sarcoidosis (EOS). Recent studies have demonstrated that EOS and Blau syndrome (juvenile systemic granulomatosis or familial/paediatric granulomatous arthritis) represent a single disease entity caused by heterozygous mutations in the NOD2 (nucleotide-binding oligomerization domain 2) gene.