Skip to main content

Neuroinflammatory Disease in Association with Morphea (Localized Scleroderma)

  • Chapter
  • First Online:
  • 786 Accesses

Abstract

This chapter describes the case of a girl with a history of localized scleroderma (LS) who developed chronic and progressive seizures which were associated with focal brain lesions and chronic progression of the brain lesions. Localized scleroderma (LS) is an uncommon autoimmune condition of the skin that may be associated with head and facial lesions in one-fourth and neurological complications in one-fifth of those with head/facial lesions. Brain MRI may show atrophy or focal T2 hyperintensities on the side ipsilateral to the skin changes. Progression of brain lesions may occur insidiously. While no randomized control trials have been performed on immunotherapy for this condition, methotrexate, steroids, cyclophosphamide, mycophenolate mofetil, and azathioprine have been tried and may be effective.

This is a preview of subscription content, log in via an institution.

Buying options

Chapter
USD   29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD   79.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD   99.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD   129.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Learn about institutional subscriptions

References

  1. Torok KS. Pediatric scleroderma: systemic or localized forms. Pediatr Clin North Am. 2012;59:381–405.

    Article  PubMed  PubMed Central  Google Scholar 

  2. Zulian F, Athreya BH, Laxer R, Nelson AM, Feitosa de Oliveira SK, Punaro MG, Cuttica R, Higgins GC, Van Suijlekom-Smit LW, Moore TL, Lindsley C, Garcia-Consuegra J, Esteves Hilario MO, Lepore L, Silva CA, Machado C, Garay SM, Uziel Y, Martini G, Foeldvari I, Peserico A, Woo P, Harper J. Juvenile localized scleroderma: clinical and epidemiological features in 750 children. An international study. Rheumatology (Oxford). 2006;45:614–20.

    Article  CAS  Google Scholar 

  3. Zulian F, Vallongo C, Woo P, Russo R, Ruperto N, Harper J, Espada G, Corona F, Mukamel M, Vesely R, Musiej-Nowakowska E, Chaitow J, Ros J, Apaz MT, Gerloni V, Mazur-Zielinska H, Nielsen S, Ullman S, Horneff G, Wouters C, Martini G, Cimaz R, Laxer R, Athreya BH. Localized scleroderma in childhood is not just a skin disease. Arthritis Rheum. 2005;52:2873–81.

    Article  PubMed  Google Scholar 

  4. Appenzeller S, Montenegro MA, Dertkigil SS, Sampaio-Barros PD, Marques-Neto JF, Samara AM, Andermann F, Cendes F. Neuroimaging findings in scleroderma en coup de sabre. Neurology. 2004;62:1585–9.

    Article  CAS  PubMed  Google Scholar 

  5. Grosso S, Fioravanti A, Biasi G, Conversano E, Marcolongo R, Morgese G, Balestri P. Linear scleroderma associated with progressive brain atrophy. Brain Dev. 2003;25:57–61.

    Article  PubMed  Google Scholar 

  6. Amaral TN, Peres FA, Lapa AT, Marques-Neto JF, Appenzeller S. Neurologic involvement in scleroderma: a systematic review. Semin Arthritis Rheum. 2013;43:335–47.

    Article  PubMed  Google Scholar 

  7. Li SC, Torok KS, Pope E, Dedeoglu F, Hong S, Jacobe HT, Rabinovich CE, Laxer RM, Higgins GC, Ferguson PJ, Lasky A, Baszis K, Becker M, Campillo S, Cartwright V, Cidon M, Inman CJ, Jerath R, O’Neil KM, Vora S, Zeft A, Wallace CA, Ilowite NT, Fuhlbrigge RC. Development of consensus treatment plans for juvenile localized scleroderma: a roadmap toward comparative effectiveness studies in juvenile localized scleroderma. Arthritis Care Res. 2012;64:1175–85.

    CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to E. Ann Yeh M.D. .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2017 Springer International Publishing AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Yeh, E.A., Weinstock-Guttman, B. (2017). Neuroinflammatory Disease in Association with Morphea (Localized Scleroderma). In: Waubant, E., Lotze, T. (eds) Pediatric Demyelinating Diseases of the Central Nervous System and Their Mimics. Springer, Cham. https://doi.org/10.1007/978-3-319-61407-6_12

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-61407-6_12

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-61405-2

  • Online ISBN: 978-3-319-61407-6

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics