Autoimmune Skin Disorders (Collagen Vascular)

  • Clayton Sontheimer
  • Heather A. Brandling-BennettEmail author


Autoimmune connective tissue diseases are frequently in the differential diagnosis when patients present with cutaneous eruptions and systemic complaints. Systemic-onset juvenile idiopathic arthritis, systemic lupus erythematosus, and juvenile dermatomyositis are three autoimmune disorders that may have prominent cutaneous findings. Recognition of the characteristic skin manifestations may be critical in ordering the appropriate workup and making the correct diagnosis.


Autoimmune Systemic-onset juvenile idiopathic arthritis Still’s disease Systemic lupus erythematosus Juvenile dermatomyositis 


  1. 1.
    Sawhney S, Woo P, Murray KJ. Macrophage activation syndrome: a potentially fatal complication of rheumatic disorders. Arch Dis Child. 2001;85(5):421–6. Epub 2001/10/23.CrossRefPubMedPubMedCentralGoogle Scholar
  2. 2.
    Stephan JL, Kone-Paut I, Galambrun C, Mouy R, Bader-Meunier B, Prieur AM. Reactive haemophagocytic syndrome in children with inflammatory disorders. A retrospective study of 24 patients. Rheumatology. 2001;40(11):1285–92. Epub 2001/11/16.CrossRefPubMedGoogle Scholar
  3. 3.
    Zeft A, Hollister R, LaFleur B, Sampath P, Soep J, McNally B, et al. Anakinra for systemic juvenile arthritis: the Rocky Mountain experience. J Clin Rheumatol. 2009;15(4):161–4. Epub 2009/04/14.CrossRefPubMedGoogle Scholar
  4. 4.
    Lequerre T, Quartier P, Rosellini D, Alaoui F, De Bandt M, Mejjad O, et al. Interleukin-1 receptor antagonist (anakinra) treatment in patients with systemic-onset juvenile idiopathic arthritis or adult onset Still disease: preliminary experience in France. Ann Rheum Dis. 2008;67(3):302–8. Epub 2007/10/20.CrossRefPubMedGoogle Scholar
  5. 5.
    Yokota S, Imagawa T, Mori M, Miyamae T, Aihara Y, Takei S, et al. Efficacy and safety of tocilizumab in patients with systemic-onset juvenile idiopathic arthritis: a randomised, double-blind, placebo-controlled, withdrawal phase III trial. Lancet. 2008;371(9617):998–1006. Epub 2008/03/25.CrossRefPubMedGoogle Scholar
  6. 6.
    De Benedetti F, Brunner HI, Ruperto N, Kenwright A, Wright S, Calvo I, et al. Randomized trial of tocilizumab in systemic juvenile idiopathic arthritis. N Engl J Med. 2012;367(25):2385–95. Epub 2012/12/21.CrossRefPubMedGoogle Scholar
  7. 7.
    Petri M, Orbai AM, Alarcon GS, Gordon C, Merrill JT, Fortin PR, et al. Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus. Arthritis Rheum. 2012;64(8):2677–86. Epub 2012/05/04.CrossRefPubMedPubMedCentralGoogle Scholar
  8. 8.
    Mina R, Brunner HI. Pediatric lupus—are there differences in presentation, genetics, response to therapy, and damage accrual compared with adult lupus? Rheum Dis Clin North Am. 2010;36(1):53–80, vii–viii. Epub 2010/03/06.CrossRefPubMedPubMedCentralGoogle Scholar
  9. 9.
    Gerami P, Walling HW, Lewis J, Doughty L, Sontheimer RD. A systematic review of juvenile-onset clinically amyopathic dermatomyositis. Br J Dermatol. 2007;157(4):637–44. Epub 2007/06/29.CrossRefPubMedGoogle Scholar
  10. 10.
    Sallum AM, Pivato FC, Doria-Filho U, Aikawa NE, Liphaus BL, Marie SK, et al. Risk factors associated with calcinosis of juvenile dermatomyositis. J Pediatr. 2008;84(1):68–74. Epub 2008/01/11.CrossRefGoogle Scholar
  11. 11.
    Tomasova Studynkova J, Charvat F, Jarosova K, Vencovsky J. The role of MRI in the assessment of polymyositis and dermatomyositis. Rheumatology. 2007;46(7):1174–9. Epub 2007/05/15.CrossRefPubMedGoogle Scholar

Copyright information

© Springer International Publishing Switzerland 2016

Authors and Affiliations

  • Clayton Sontheimer
    • 1
  • Heather A. Brandling-Bennett
    • 2
    Email author
  1. 1.Division of Rheumatology, Department of PediatricsSeattle Children’s Hospital/University of WashingtonSeattleUSA
  2. 2.Division of Dermatology, Department of PediatricsSeattle Children’s Hospital/University of WashingtonSeattleUSA

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