Skip to main content
SpringerLink
Log in

Raynaud Phenomenon in the Pediatric Age

  • Chapter
  • First Online:
Raynaud’s Phenomenon

  • 1615 Accesses

Abstract

Raynaud phenomenon (RP) in children may, as in adults, be primary (idiopathic) or secondary to rheumatic diseases, drugs, or other conditions. This chapter aims to present a practical approach to clinical assessment and treatment of children with RP, with supporting evidence from studies specific to the pediatric age group. As with adults, RP can be the presenting feature of connective tissue disease (CTD). However, risk factors for progression from RP symptoms to a defined CTD phenotype are less well defined in children. The differentiation between primary and secondary forms of RP is important. Those with primary RP have a good outcome and can be managed with non-pharmacological approaches and, where necessary, vasodilatory drugs, with reassurance to the child and family that this condition is relatively benign. Identification of a child with secondary RP or at high risk to progression to secondary RP based on history, examination, and investigations is important to allow more careful monitoring and follow-up, with the aim to detect evolving CTD early.

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

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 89.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 119.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 169.99
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

Institutional subscriptions

Abbreviations

ANA:

Antinuclear antibody

CCBs:

Calcium channel blockers

CRPS:

Complex regional pain syndrome

CTD:

Connective tissue disease

JSSc:

Juvenile systemic sclerosis

LDI:

Laser Doppler imaging

NFC:

Nailfold capillaroscopy

RP:

Raynaud phenomenon

SSc:

Systemic sclerosis

References

  1. LeRoy EC, Medsger Jr TA. Raynaud’s phenomenon: a proposal for classification. Clin Exp Rheumatol. 1992;10(5):485–8.

    PubMed  CAS  Google Scholar 

  2. Nigrovic PA, Fuhlbrigge RC, Sundel RP. Raynaud’s phenomenon in children: a retrospective review of 123 patients. Pediatrics. 2003;111(4 Pt 1):715–21.

    Article  PubMed  Google Scholar 

  3. Pavlov-Dolijanovic S, Damjanov N, Ostojic P, Susic G, Stojanovic R, Gacic D, et al. The prognostic value of nailfold capillary changes for the development of connective tissue disease in children and adolescents with primary Raynaud phenomenon: a follow-up study of 250 patients. Pediatr Dermatol. 2006;23(5):437–42.

    Article  PubMed  Google Scholar 

  4. Piotto DG, Hilario MO, Carvalho NS, Len CA, Andrade LE, Terreri MT. Prospective nailfold capillaroscopy evaluation of Raynaud’s phenomenon in children and adolescents. Acta Reumatol Port. 2013;38(2):114–21.

    PubMed  Google Scholar 

  5. Jones GT, Herrick AL, Woodham SE, Baildam EM, Macfarlane GJ, Silman AJ. Occurrence of Raynaud’s phenomenon in children ages 12-15 years: prevalence and association with other common symptoms. Arthritis Rheum. 2003;48(12):3518–21.

    Article  PubMed  Google Scholar 

  6. Herrick AL. The pathogenesis, diagnosis and treatment of Raynaud phenomenon. Nat Rev Rheumatol. 2012;8(8):469–79.

    Article  PubMed  CAS  Google Scholar 

  7. Herrick AL, Jayson MI. Primary Raynaud’s phenomenon in early childhood. Br J Rheumatol. 1991;30(3):223–5.

    Article  PubMed  CAS  Google Scholar 

  8. Sharathkumar AA, Castillo-Caro P. Primary Raynaud’s phenomenon in an infant: a case report and review of literature. Pediatr Rheumatol Online J. 2011;9:16.

    Article  PubMed  PubMed Central  Google Scholar 

  9. Shouval DS, Mukamel M, Zulian F, Amir J, Harel L. Iloprost treatment for refractory Raynaud’s phenomenon in two infants. Clin Exp Rheumatol. 2008;26(3 Suppl 49):S105–7.

    PubMed  CAS  Google Scholar 

  10. Martini G, Foeldvari I, Russo R, Cuttica R, Eberhard A, Ravelli A, et al. Systemic sclerosis in childhood: clinical and immunologic features of 153 patients in an international database. Arthritis Rheum. 2006;54(12):3971–8.

    Article  PubMed  CAS  Google Scholar 

  11. Russo RA, Katsicas MM. Clinical characteristics of children with Juvenile Systemic Sclerosis: follow-up of 23 patients in a single tertiary center. Pediatr Rheumatol Online J. 2007;5:6.

    Article  PubMed  PubMed Central  Google Scholar 

  12. Scalapino K, Arkachaisri T, Lucas M, Fertig N, Helfrich DJ, Londino Jr AV, et al. Childhood onset systemic sclerosis: classification, clinical and serologic features, and survival in comparison with adult onset disease. J Rheumatol. 2006;33(5):1004–13.

    PubMed  Google Scholar 

  13. Hawley DP, Baildam EM, Amin TS, Cruikshank MK, Davidson JE, Dixon J, et al. Access to care for children and young people diagnosed with localized scleroderma or juvenile SSc in the UK. Rheumatology (Oxford). 2012;51(7):1235–9.

    Article  Google Scholar 

  14. Foeldvari I, Nihtyanova SI, Wierk A, Denton CP. Characteristics of patients with juvenile onset systemic sclerosis in an adult single-center cohort. J Rheumatol. 2010;37(11):2422–6.

    Article  PubMed  Google Scholar 

  15. Zulian F, Vallongo C, Woo P, Russo R, Ruperto N, Harper J, et al. Localized scleroderma in childhood is not just a skin disease. Arthritis Rheum. 2005;52(9):2873–81.

    Article  PubMed  Google Scholar 

  16. Christen-Zaech S, Hakim MD, Afsar FS, Paller AS. Pediatric morphea (localized scleroderma): review of 136 patients. J Am Acad Dermatol. 2008;59(3):385–96.

    Article  PubMed  Google Scholar 

  17. Tsai YY, Yang YH, Yu HH, Wang LC, Lee JH, Chiang BL. Fifteen-year experience of pediatric-onset mixed connective tissue disease. Clin Rheumatol. 2010;29(1):53–8.

    Article  PubMed  Google Scholar 

  18. Chiechengchol D, Murphy R, Edwards SW, Leone V, Friswell M, Pilkington C, et al. Mucocutaneous manifestations in a national cohort of juvenile-onset systemic lupus erythematosus. Rheumatology (Oxford). 2014;53(8):1504–12.

    Google Scholar 

  19. Cimaz R, Casadei A, Rose C, Bartunkova J, Sediva A, Falcini F, et al. Primary Sjogren syndrome in the paediatric age: a multicentre survey. Eur J Pediatr. 2003;162(10):661–5.

    Article  PubMed  Google Scholar 

  20. Goldman W, Seltzer R, Reuman P. Association between treatment with central nervous system stimulants and Raynaud’s syndrome in children: a retrospective case-control study of rheumatology patients. Arthritis Rheum. 2008;58(2):563–6.

    Article  PubMed  Google Scholar 

  21. Ingegnoli F, Herrick AL. Nailfold capillaroscopy in pediatrics. Arthritis Care Res (Hoboken). 2013;65(9):1393–400.

    Article  Google Scholar 

  22. Tudor A, Musat A, Teleianu C, Zapucioiu C, Bari M, Comanici A. Aspects of nailfold capillaroscopy in children and young people with rheumatic disease. Rom J Intern Med. 2004;42(1):211–5.

    PubMed  Google Scholar 

  23. Dolezalova P, Young SP, Bacon PA, Southwood TR. Nailfold capillary microscopy in healthy children and in childhood rheumatic diseases: a prospective single blind observational study. Ann Rheum Dis. 2003;62(5):444–9.

    Article  PubMed  CAS  PubMed Central  Google Scholar 

  24. Ingegnoli F, Zeni S, Gerloni V, Fantini F. Capillaroscopic observations in childhood rheumatic diseases and healthy controls. Clin Exp Rheumatol. 2005;23(6):905–11.

    PubMed  CAS  Google Scholar 

  25. Terreri MT, Andrade LE, Puccinelli ML, Hilario MO, Goldenberg J. Nail fold capillaroscopy: normal findings in children and adolescents. Semin Arthritis Rheum. 1999;29(1):36–42.

    Article  PubMed  CAS  Google Scholar 

  26. Christen-Zaech S, Seshadri R, Sundberg J, Paller AS, Pachman LM. Persistent association of nailfold capillaroscopy changes and skin involvement over thirty-six months with duration of untreated disease in patients with juvenile dermatomyositis. Arthritis Rheum. 2008;58(2):571–6.

    Article  PubMed  PubMed Central  Google Scholar 

  27. Nascif AK, Terreri MT, Len CA, Andrade LE, Hilario MO. Inflammatory myopathies in childhood: correlation between nailfold capillaroscopy findings and clinical and laboratory data. J Pediatr (Rio J). 2006;82(1):40–5.

    Article  Google Scholar 

  28. Ostrowski RA, Sullivan CL, Seshadri R, Morgan GA, Pachman LM. Association of normal nailfold end row loop numbers with a shorter duration of untreated disease in children with juvenile dermatomyositis. Arthritis Rheum. 2010;62(5):1533–8.

    Article  PubMed  PubMed Central  Google Scholar 

  29. Murray AK, Moore TL, Manning JB, Taylor C, Griffiths CE, Herrick AL. Noninvasive imaging techniques in the assessment of scleroderma spectrum disorders. Arthritis Rheum. 2009;61(8):1103–11.

    Article  PubMed  Google Scholar 

  30. Jayanetti S, Smith CP, Moore T, Jayson MI, Herrick AL. Thermography and nailfold capillaroscopy as noninvasive measures of circulation in children with Raynaud’s phenomenon. J Rheumatol. 1998;25(5):997–9.

    PubMed  CAS  Google Scholar 

  31. Biernacka-Zielinska M, Brozik H, Smolewska E, Mikinka M, Jakubowska T, Stanczyk J. [Diagnostic value of thermography and endothelin concentration in serum of children with Raynaud’s Syndrome]. Med Wieku Rozwoj. 2005;9(2):213–22.

    PubMed  Google Scholar 

  32. Piotto DG, Correa MJ. Miotto e Silva VB, Kayser C, Terreri MT. Laser Doppler imaging for assessment of microcirculation in juvenile systemic sclerosis. Rheumatology (Oxford). 2014;53(1):72–5.

    Article  Google Scholar 

  33. Duffy CM, Laxer RM, Lee P, Ramsay C, Fritzler M, Silverman ED. Raynaud syndrome in childhood. J Pediatr. 1989;114(1):73–8.

    Article  PubMed  CAS  Google Scholar 

  34. Cooke JP, Marshall JM. Mechanisms of Raynaud’s disease. Vasc Med. 2005;10(4):293–307.

    Article  PubMed  Google Scholar 

  35. Schulze UM, Pettke-Rank CV, Kreienkamp M, Hamm H, Brocker EB, Wewetzer C, et al. Dermatologic findings in anorexia and bulimia nervosa of childhood and adolescence. Pediatr Dermatol. 1999;16(2):90–4.

    Article  PubMed  CAS  Google Scholar 

  36. Rustin MH, Foreman JC, Dowd PM. Anorexia nervosa associated with acromegaloid features, onset of acrocyanosis and Raynaud’s phenomenon and worsening of chilblains. J R Soc Med. 1990;83(8):495–6.

    PubMed  CAS  PubMed Central  Google Scholar 

  37. Haveri FT, Inamadar AC. A cross-sectional prospective study of cutaneous lesions in newborn. ISRN Dermatol. 2014;2014:360590.

    PubMed  PubMed Central  Google Scholar 

  38. Ashurst J, Wasson M. Methemoglobinemia: a systematic review of the pathophysiology, detection, and treatment. Del Med J. 2011;83(7):203–8.

    PubMed  Google Scholar 

  39. Padeh S, Gerstein M, Greenberger S, Berkun Y. Chronic chilblains: the clinical presentation and disease course in a large paediatric series. Clin Exp Rheumatol. 2013;31(3):463–8.

    PubMed  Google Scholar 

  40. Larkins N, Murray KJ. Major cluster of chilblain cases in a cold dry Western Australian winter. J Paediatr Child Health. 2013;49(2):144–7.

    Article  PubMed  Google Scholar 

  41. Lamberti PM, Light TR. Carpal tunnel syndrome in children. Hand Clin. 2002;18(2):331–7.

    Article  PubMed  Google Scholar 

  42. Cook-Norris RH, Toffelson MM, Cruz-Inigo AE, Sandroni P, Davis MD, Davis DM. Pediatric erythromelalgia: a retrospective review of 32 cases evaluated at Mayo Clinic over a 37-year period. J Am Acad Dermatol. 2012;66(3):416–23.

    Article  PubMed  Google Scholar 

  43. Dib-Hajj SD, Yang Y, Waxman SG. Genetics and molecular pathophysiology of Na(v)1.7-related pain syndromes. Adv Genet. 2008;63:85–110.

    Article  PubMed  CAS  Google Scholar 

  44. Layzer RB. Hot feet: erythromelalgia and related disorders. J Child Neurol. 2001;16(3):199–202.

    Article  PubMed  CAS  Google Scholar 

  45. Kachko L, Efrat R, Ben Ami S, Mukamel M, Katz J. Complex regional pain syndromes in children and adolescents. Pediatr Int. 2008;50(4):523–7.

    Article  PubMed  Google Scholar 

  46. Chan KH, Gitomer SA, Perkins JN, Liang C, Strain JD. Clinical presentation of cervical ribs in the pediatric population. J Pediatr. 2013;162(3):635–6.

    Article  PubMed  Google Scholar 

  47. Gargh K, Baildam EM, Cleary GA, Beresford MW, McCann LJ. A retrospective clinical analysis of pharmacological modalities used for symptomatic relief of Raynaud’s phenomenon in children treated in a UK paediatric rheumatology centre. Rheumatology (Oxford). 2010;49(1):193–4.

    Article  Google Scholar 

  48. Kaya IS, Senses DA, Dilmen U. Nifedipine in the treatment of Raynaud’s disease in childhood. Lancet. 1989;1(8647):1136.

    Article  PubMed  CAS  Google Scholar 

  49. Burns EC, Dunger DB, Dillon MJ. Raynaud’s disease. Arch Dis Child. 1985;60(6):537–41.

    Article  PubMed  CAS  PubMed Central  Google Scholar 

  50. Dunger DB, Dillon MJ, Daman-Willems C, Cooke ED, Bowcock SA. Treatment of childhood Raynaud’s disease by transdermal prostaglandin E2 analogue. Lancet. 1985;2(8445):50.

    Article  PubMed  CAS  Google Scholar 

  51. Zulian F, Corona F, Gerloni V, Falcini F, Buoncompagni A, Scarazatti M, et al. Safety and efficacy of iloprost for the treatment of ischaemic digits in paediatric connective tissue diseases. Rheumatology (Oxford). 2004;43(2):229–33.

    Article  CAS  Google Scholar 

  52. Roldan T, Deiros L, Romero JA, Gutierrez-Larraya F, Herrero A, Del Cerro MJ. Safety and tolerability of targeted therapies for pulmonary hypertension in children. Pediatr Cardiol. 2014;35(3):490–8.

    Article  PubMed  CAS  Google Scholar 

  53. Shimizu M, Hashida Y, Ueno K, Yokoyama T, Nakayama Y, Saito T, et al. Successful treatment with bosentan for pulmonary hypertension and reduced peripheral circulation in juvenile systemic sclerosis. Pediatr Cardiol. 2011;32(7):1040–2.

    Article  PubMed  Google Scholar 

  54. Wahezi DM, Ilowite NT, Kenney-Riley KM, Belamarich PF. Chronic leg ulceration as the presenting feature of diffuse systemic sclerosis in childhood. J Pediatr. 2011;159(4):698.

    Article  PubMed  Google Scholar 

  55. Gonzalez-Fernandez MA, Garcia-Consuegra J. Polyarteritis nodosa resistant to conventional treatment in a pediatric patient. Ann Pharmacother. 2007;41(5):885–90.

    Article  PubMed  CAS  Google Scholar 

  56. Williams VL, Guirola R, Flemming K, Modi GM, Rosales C, DeGuzman MM. Distal extremity necrosis as a manifestation of cutaneous polyarteritis nodosa: case report and review of the acute management of a pediatric patient. Pediatr Dermatol. 2012;29(4):473–8.

    Article  PubMed  Google Scholar 

  57. Drake DB, Kesler RW, Morgan RF. Digital sympathectomy for refractory Raynaud’s phenomenon in an adolescent. J Rheumatol. 1992;19(8):1286–8.

    PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Pediatric Rheumatology Unit, Department of Pediatrics, University of Padua, Via Giustiniani 3, 35128, Padua, Italy

    Francesco Zulian

  2. Department of Paediatric Rheumatology, Alder Hey Children’s NHS Foundation Trust, Liverpool, UK

    Clare Pain

Authors
  1. Francesco Zulian
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Clare Pain
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Francesco Zulian .

Editor information

Editors and Affiliations

  1. School of Medicine, Johns Hopkins University, Baltimore, Maryland, USA

    Fredrick M. Wigley

  2. Department of Rheumatology, The University of Manchester, Manchester, United Kingdom

    Ariane L. Herrick

  3. Department of Anesthesiology and Critical Care Medicine, Johns Hopkins University School of Medic, Baltimore, Maryland, USA

    Nicholas A. Flavahan

Rights and permissions

Reprints and permissions

Copyright information

© 2015 Springer Science+Business Media New York

About this chapter

Cite this chapter

Zulian, F., Pain, C. (2015). Raynaud Phenomenon in the Pediatric Age. In: Wigley, F., Herrick, A., Flavahan, N. (eds) Raynaud’s Phenomenon. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-1526-2_7

Download citation

  • DOI: https://doi.org/10.1007/978-1-4939-1526-2_7

  • Published:

  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4939-1525-5

  • Online ISBN: 978-1-4939-1526-2

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation