164. Erythema Elevatum Diutinum

  1. Alan D. Irvine MD, FRCPI, FRCP3,4,
  2. Peter H. Hoeger MD5,6 and
  3. Albert C. Yan MD, FAAP, FAAD7,8
  1. Aimee C. Smidt MD1 and
  2. Sarah L. Chamlin MD2

Published Online: 24 MAY 2011

DOI: 10.1002/9781444345384.ch164

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

Smidt, A. C. and Chamlin, S. L. (2011) Erythema Elevatum Diutinum, 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.ch164

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

    Departments of Dermatology and Pediatrics, University of New Mexico School of Medicine, Albuquerque, NM, USA

  2. 2

    Departments of Pediatrics and Dermatology, Northwestern University, Feinberg School of Medicine, Children's Memorial Hospital, Chicago, IL, USA

Publication History

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

ISBN Information

Print ISBN: 9781405176958

Online ISBN: 9781444345384

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Keywords:

  • Small-vessel vasculitis;
  • Neutrophilic dermatosis;
  • Immune-complex mediated;
  • Streptococcal infection;
  • HIV;
  • Paraproteinemia;
  • Dapsone

Summary

Erythema elevatum diutinum (EED) is a rare type of localized cutaneous vasculitis, characterized by a chronic, fibrosing course. It typically presents with symmetric, red-brown papules and plaques overlying joints and extensor surfaces. Most cases are asymptomatic, though pruritus, burning and tenderness have been described. The exact cause of EED is unknown, but it is thought to represent a form of immune-complex mediated small vessel vasculitis, possibly induced by chronic antigen exposure. Known associations include: infections, most notably streptococcus and HIV, autoimmune disease, celiac disease, inflammatory bowel disease, and hematologic abnormalities, among others. Treatment involves addressing the underlying etiology if possible, and first-line therapy includes dapsone or other sulfonamides.