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Pediatric Raynaud Phenomenon Treatment & Management

  • Author: Suzanne C Li, MD, PhD; Chief Editor: Lawrence K Jung, MD  more...
 
Updated: Apr 22, 2014
 

Medical Care

See the list below:

  • Patients with Raynaud phenomenon (RP) should avoid situations and stressors that aggravate the disease (eg, vibration, cold exposure, digital trauma).[5]
  • Patients should avoid medications and drugs that aggravate the disease (eg, smoking, cocaine, other medications listed above).
  • Patients need to adequately cover themselves in cold weather by covering core areas (head, trunk) and extremities using hats, layers, mittens, thick socks, and boots.
  • Patients should come in from the cold when they feel a change in digit sensation or when prolonged pallor or cyanosis of the extremities occurs.
  • Digital wounds should be carefully treated and monitored. Clean wounds twice daily with soap and water, cover with a topical antibiotic ointment (eg, bacitracin, mupirocin [Bactroban]) and then with a light bandage.[5] Systemic antibiotics may be required if the infection worsens. Digital ulceration should be treated in a similar fashion.[5]
  • The feet should be evaluated for any problems; approximately 10% of adult patients with systemic sclerosis have foot ulcers.[7]
  • Biofeedback was reported to enable a pediatric patient to raise her digit temperature by 12o C and to aid some adult patients.[1, 17] Trials of biofeedback have yielded mixed results with some reporting benefits and others not. Part of the issue may be that biofeedback can be slow to learn so lack of efficacy may be partly related to patients failing to learn to do it effectively.[7, 5, 86, 87]
  • Auricular electroacupuncture was reported to decrease the frequency and severity of attacks in primary Raynaud phenomenon patients in an uncontrolled study.[88]
  • Low-level laser irradiation has also been reported to significantly reduce the frequency and severity of attacks in primary Raynaud phenomenon and secondary Raynaud phenomenon compared with sham irradiation.[89]
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Surgical Care

Surgical care is needed for serious morbidity problems, including the following[7, 90] :

  • Debridement of necrotic tissue, including infected wounds and osteomyelitis, may be necessary.
  • Amputation of gangrenous digit may be required.
  • Different surgical techniques have been used to improve Raynaud phenomenon symptoms, including balloon angioplasty, venous or arterial grafting, and digital or thorascopic sympathectomy.[91, 87] Sympathetic block can help improve blood flow and is useful for threatened severe digit ischemia.[32]
  • Sympathectomy may be required for intractable ischemia not responsive to medical treatment. Digital artery sympathectomy is more commonly advised compared with cervical sympathectomy because of lower morbidity.[7, 92]
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Consultations

See the list below:

  • A pediatric rheumatologist should be consulted to evaluate patients suspected of having or developing secondary Raynaud phenomenon.
  • Other subspecialists, such as a hematologist, oncologist, or endocrinologist, should be consulted if the underlying condition for Raynaud phenomenon falls in their domain.
  • Consultation with a surgeon may be needed for patients with threatened digit ischemia.
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Diet

See the list below:

  • No specific diet recommendations are necessary.
  • Supplements such as fish oil and evening primrose have been reported to be beneficial in limited studies, but additional trials are needed for better evaluation of the effectiveness of these products.[32, 93] In placebo-controlled trials, neither gingko biloba nor St. John’s Wort were found to provide any significant efficacy.[90]
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Activity

See the list below:

  • Patients should carefully monitor symptoms in situations that may precipitate an episode.
  • If the patient is involved in winter sports, limiting the duration of continuous cold exposure, wearing layers, and using hand warmers may help.
  • In the spring and fall seasons, outdoor activity may also precipitate episodes during damp or rainy days.[9]
  • Patients who participate in activities that may result in digital injury (eg, skateboarding, mountain biking) should wear protective gloves to minimize abrasions and deeper injuries.
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Contributor Information and Disclosures
Author

Suzanne C Li, MD, PhD Associate Professor, Department of Pediatrics, Rutgers New Jersey Medical School; Senior Attending, Department of Pediatrics, Joseph M Sanzari Children’s Hospital, Hackensack University Medical Center

Suzanne C Li, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, American College of Rheumatology, Childhood Arthritis and Rheumatology Research Alliance

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

David D Sherry, MD Chief, Rheumatology Section, Director, Amplified Musculoskeletal Pain Program, The Children's Hospital of Philadelphia; Professor of Pediatrics, University of Pennsylvania School of Medicine

David D Sherry, MD is a member of the following medical societies: American College of Rheumatology, American Pain Society

Disclosure: Nothing to disclose.

Chief Editor

Lawrence K Jung, MD Chief, Division of Pediatric Rheumatology, Children's National Medical Center

Lawrence K Jung, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Rheumatology, Clinical Immunology Society, New York Academy of Sciences

Disclosure: Nothing to disclose.

Additional Contributors

James M Oleske, MD, MPH François-Xavier Bagnoud Professor of Pediatrics, Director, Division of Pulmonary, Allergy, Immunology and Infectious Diseases, Department of Pediatrics, Rutgers New Jersey Medical School; Professor, Department of Quantitative Methods, Rutgers New Jersey Medical School

James M Oleske, MD, MPH is a member of the following medical societies: Academy of Medicine of New Jersey, American Academy of Allergy Asthma and Immunology, American Academy of Hospice and Palliative Medicine, American Association of Public Health Physicians, American College of Preventive Medicine, American Pain Society, Infectious Diseases Society of America, Infectious Diseases Society of New Jersey, Medical Society of New Jersey, Pediatric Infectious Diseases Society, Arab Board of Family Medicine, American Academy of Pain Management, National Association of Pediatric Nurse Practitioners, Association of Clinical Researchers and Educators, American Academy of HIV Medicine, American Thoracic Society, American Academy of Pediatrics, American Public Health Association, American Society for Microbiology, Infectious Diseases Society of America, Pediatric Infectious Diseases Society

Disclosure: Nothing to disclose.

References
  1. Athreya BH. Vasospastic disorders in children. Semin Pediatr Surg. 1994 May. 3(2):70-8. [Medline].

  2. Wigley FM. Clinical practice. Raynaud's Phenomenon. N Engl J Med. 2002 Sep 26. 347(13):1001-8. [Medline].

  3. Current world literature. Curr Opin Rheumatol. 2008 Nov. 20(6):729-35. [Medline].

  4. Prete M, Fatone MC, Favoino E, Perosa F. Raynaud's phenomenon: From molecular pathogenesis to therapy. Autoimmun Rev. 2014 Jun. 13(6):655-667. [Medline].

  5. Hummers LK, Wigley FM. Management of Raynaud's phenomenon and digital ischemic lesions in scleroderma. Rheum Dis Clin North Am. 2003 May. 29(2):293-313. [Medline].

  6. Nigrovic PA, Fuhlbrigge RC, Sundel RP. Raynaud's phenomenon in children: a retrospective review of 123 patients. Pediatrics. 2003 Apr. 111(4 Pt 1):715-21. [Medline].

  7. Herrick AL. Treatment of Raynaud's phenomenon: new insights and developments. Curr Rheumatol Rep. 2003 Apr. 5(2):168-74. [Medline].

  8. Fonseca C, Abraham D, Ponticos M. Neuronal regulators and vascular dysfunction in Raynaud's phenomenon and systemic sclerosis. Curr Vasc Pharmacol. 2009 Jan. 7(1):34-9. [Medline].

  9. Pope JE. The diagnosis and treatment of Raynaud's phenomenon: a practical approach. Drugs. 2007. 67(4):517-25. [Medline].

  10. Hirschl M, Hirschl K, Lenz M, Katzenschlager R, Hutter HP, Kundi M. Transition from primary Raynaud's phenomenon to secondary Raynaud's phenomenon identified by diagnosis of an associated disease: results of ten years of prospective surveillance. Arthritis Rheum. 2006 Jun. 54(6):1974-81. [Medline].

  11. Al-Allaf A, Belch J. M. Hochberg, A. Silman, J. Smolen, M. Weinblatt and M. Weisman. Raynaud's phenomenon. Rheumatology. Philadelphia: Mosby; 2003. 2: 1507-12.

  12. Herrick AL. Pathogenesis of Raynaud's phenomenon. Rheumatology (Oxford). 2005 May. 44(5):587-96. [Medline].

  13. Swart JF, Wulffraat NM. Diagnostic workup for mixed connective tissue disease in childhood. Isr Med Assoc J. 2008 Aug-Sep. 10(8-9):650-2. [Medline].

  14. Burns EC, Dunger DB, Dillon MJ. Raynaud's disease. Arch Dis Child. 1985 Jun. 60(6):537-41. [Medline].

  15. Duffy CM, Laxer RM, Lee P, Ramsay C, Fritzler M, Silverman ED. Raynaud syndrome in childhood. J Pediatr. 1989 Jan. 114(1):73-8. [Medline].

  16. LeRoy EC, Medsger TA Jr. Raynaud's phenomenon: a proposal for classification. Clin Exp Rheumatol. 1992 Sep-Oct. 10(5):485-8. [Medline].

  17. Bolster MB, Maricq HR, Leff RL. Office evaluation and treatment of Raynaud's phenomenon. Cleve Clin J Med. 1995 Jan-Feb. 62(1):51-61. [Medline].

  18. Turton EP, Kent PJ, Kester RC. The aetiology of Raynaud's phenomenon. Cardiovasc Surg. 1998 Oct. 6(5):431-40. [Medline].

  19. Pavlov-Dolijanovic S, Damjanov NS, Vujasinovic Stupar NZ, Radunovic GL, Stojanovic RM, Babic D. Late appearance and exacerbation of primary Raynaud's phenomenon attacks can predict future development of connective tissue disease: a retrospective chart review of 3,035 patients. Rheumatol Int. 2013 Apr. 33(4):921-6. [Medline].

  20. Flavahan NA, Flavahan S, Mitra S, Chotani MA. The vasculopathy of Raynaud's phenomenon and scleroderma. Rheum Dis Clin North Am. 2003 May. 29(2):275-91, vi. [Medline].

  21. Chung L, Utz PJ. Antibodies in scleroderma: direct pathogenicity and phenotypic associations. Curr Rheumatol Rep. 2004 Apr. 6(2):156-63. [Medline].

  22. Gabrielli A, Svegliati S, Moroncini G, Amico D. New insights into the role of oxidative stress in scleroderma fibrosis. Open Rheumatol J. 2012. 6:87-95. [Medline].

  23. Herrick AL. Contemporary management of Raynaud's phenomenon and digital ischaemic complications. Curr Opin Rheumatol. 2011 Nov. 23(6):555-61. [Medline].

  24. Hottenga JJ, Vanmolkot KR, Kors EE, et al. The 3p21.1-p21.3 hereditary vascular retinopathy locus increases the risk for Raynaud's phenomenon and migraine. Cephalalgia. 2005 Dec. 25(12):1168-72. [Medline].

  25. Susol E, MacGregor AJ, Barrett JH, et al. A two-stage, genome-wide screen for susceptibility loci in primary Raynaud's phenomenon. Arthritis Rheum. 2000 Jul. 43(7):1641-6. [Medline].

  26. 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 Dec. 48(12):3518-21. [Medline].

  27. Planchon B, Pistorius MA, Beurrier P, De Faucal P. Primary Raynaud's phenomenon. Age of onset and pathogenesis in a prospective study of 424 patients. Angiology. 1994 Aug. 45(8):677-86. [Medline].

  28. Maricq HR, Carpentier PH, Weinrich MC, et al. Geographic variation in the prevalence of Raynaud's phenomenon: a 5 region comparison. J Rheumatol. 1997 May. 24(5):879-89. [Medline].

  29. Suter LG, Murabito JM, Felson DT, Fraenkel L. The incidence and natural history of Raynaud's phenomenon in the community. Arthritis Rheum. 2005 Apr. 52(4):1259-63. [Medline].

  30. Pavlov-Dolijanovic S, Damjanov N, Ostojic P, 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 Sep-Oct. 23(5):437-42. [Medline].

  31. Martini G, Foeldvari I, Russo R, et al. Systemic sclerosis in childhood: clinical and immunologic features of 153 patients in an international database. Arthritis Rheum. 2006 Dec. 54(12):3971-8. [Medline].

  32. Pope JE. The diagnosis and treatment of Raynaud's phenomenon: a practical approach. Drugs. 2007. 67(4):517-25. [Medline].

  33. Iqbal S, Sher MR, Good RA, Cawkwell GD. Diversity in presenting manifestations of systemic lupus erythematosus in children. J Pediatr. 1999 Oct. 135(4):500-5. [Medline].

  34. Singsen BH, Bernstein BH, Kornreich HK, King KK, Hanson V, Tan EM. Mixed connective tissue disease in childhood. A clinical and serologic survey. J Pediatr. 1977 Jun. 90(6):893-900. [Medline].

  35. 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 May. 62(5):444-9. [Medline].

  36. Guntheroth WG, Morgan BC, Harbinson JA, Mullins GL. Raynaud's disease in children. Circulation. 1967 Nov. 36(5):724-9. [Medline].

  37. Nigrovic PA, Fuhlbrigge RC, Sundel RP. Raynaud's phenomenon in children: a retrospective review of 123 patients. Pediatrics. 2003 Apr. 111(4 Pt 1):715-21. [Medline].

  38. Gendi NS, Welsh KI, Van Venrooij WJ, Vancheeswaran R, Gilroy J, Black CM. HLA type as a predictor of mixed connective tissue disease differentiation. Ten-year clinical and immunogenetic followup of 46 patients. Arthritis Rheum. 1995 Feb. 38(2):259-66. [Medline].

  39. Walker UA, Tyndall A, Czirjak L, et al. Clinical risk assessment of organ manifestations in systemic sclerosis: a report from the EULAR Scleroderma Trials And Research group database. Ann Rheum Dis. 2007 Jun. 66(6):754-63. [Medline].

  40. Ellis WW, Baer AN, Robertson RM, Pincus T, Kronenberg MW. Left ventricular dysfunction induced by cold exposure in patients with systemic sclerosis. Am J Med. 1986 Mar. 80(3):385-92. [Medline].

  41. Vergnon JM, Barthelemy JC, Riffat J, Boissier C, Claudy A, Emonot A. Raynaud's phenomenon of the lung. A reality both in primary and secondary Raynaud syndrome. Chest. 1992 May. 101(5):1312-7. [Medline].

  42. Tschope C, Westermann D, Steendijk P, et al. Coronary vasospasm-induced acute diastolic dysfunction in a patient with Raynaud's phenomenon. Clin Res Cardiol. 2006 Jun. 95(6):344-8. [Medline].

  43. Mizuno R, Fujimoto S, Saito Y, Nakamura S. Cardiac Raynaud's phenomenon induced by cold provocation as a predictor of long-term left ventricular dysfunction and remodelling in systemic sclerosis: 7-year follow-up study. Eur J Heart Fail. 2010 Mar. 12(3):268-75. [Medline].

  44. Maricq HR, Weinrich MC, Keil JE, LeRoy EC. Prevalence of Raynaud phenomenon in the general population. A preliminary study by questionnaire. J Chronic Dis. 1986. 39(6):423-7. [Medline].

  45. Silman A, Holligan S, Brennan P, Maddison P. Prevalence of symptoms of Raynaud's phenomenon in general practice. BMJ. 1990 Sep 22. 301(6752):590-2. [Medline].

  46. De Angelis R, Salaffi F, Grassi W. Raynaud's phenomenon: prevalence in an Italian population sample. Clin Rheumatol. 2006 Jul. 25(4):506-10. [Medline].

  47. Czirjak L, Kiss CG, Lovei C, et al. Survey of Raynaud's phenomenon and systemic sclerosis based on a representative study of 10,000 south-Transdanubian Hungarian inhabitants. Clin Exp Rheumatol. 2005 Nov-Dec. 23(6):801-8. [Medline].

  48. Maricq HR, Weinrich MC. Diagnosis of Raynaud's phenomenon assisted by color charts. J Rheumatol. 1988 Mar. 15(3):454-9. [Medline].

  49. Wollersheim H, Thien T. The diagnostic value of clinical signs and symptoms in patients with Raynaud's phenomenon. A cross-sectional study. Neth J Med. 1990 Dec. 37(5-6):171-82. [Medline].

  50. Brennan P, Silman A, Black C, et al. Validity and reliability of three methods used in the diagnosis of Raynaud's phenomenon. The UK Scleroderma Study Group. Br J Rheumatol. 1993 May. 32(5):357-61. [Medline].

  51. Chikura B, Moore T, Manning J, Vail A, Herrick AL. Thumb involvement in Raynaud's phenomenon as an indicator of underlying connective tissue disease. J Rheumatol. 2010 Apr. 37(4):783-6. [Medline].

  52. Buoncompagni A, Scribanis R, Picco P, et al. Clinical diversity of Raynaud's phenomenon in childhood: report of six cases. Lupus. 1993 Jun. 2(3):183-6. [Medline].

  53. 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 May. 25(5):997-9. [Medline].

  54. Cutolo M, Sulli A, Smith V. How to perform and interpret capillaroscopy. Best Pract Res Clin Rheumatol. 2013 Apr. 27(2):237-48. [Medline].

  55. Ingegnoli F, Boracchi P, Gualtierotti R, Lubatti C, Meani L, Zahalkova L. Prognostic model based on nailfold capillaroscopy for identifying Raynaud's phenomenon patients at high risk for the development of a scleroderma spectrum disorder: PRINCE (prognostic index for nailfold capillaroscopic examination). Arthritis Rheum. 2008 Jul. 58(7):2174-82. [Medline].

  56. Piotto DG, Len CA, Hilário MO, Terreri MT. Nailfold capillaroscopy in children and adolescents with rheumatic diseases. Rev Bras Reumatol. 2012 Oct. 52(5):722-32. [Medline].

  57. Quintero-Del-Rio AI, Punaro M, Pascual V. Faces of eosinophilic fasciitis in childhood. J Clin Rheumatol. 2002 Apr. 8(2):99-103. [Medline].

  58. Roquelaure Y, Ha C, Le Manac'h AP, Bodin J, Bodere A, Bosseau C, et al. Risk factors for Raynaud's phenomenon in the workforce. Arthritis Care Res (Hoboken). 2012 Jun. 64(6):898-904. [Medline].

  59. Brand FN, Larson MG, Kannel WB, McGuirk JM. The occurrence of Raynaud's phenomenon in a general population: the Framingham Study. Vasc Med. 1997 Nov. 2(4):296-301. [Medline].

  60. Iorio R, Spagnuolo MI, Sepe A, Zoccali S, Alessio M, Vegnente A. Severe Raynaud's phenomenon with chronic hepatis C disease treated with interferon. Pediatr Infect Dis J. 2003 Feb. 22(2):195-7. [Medline].

  61. Alonso-Ruiz A, Calabozo M, Perez-Ruiz F, Mancebo L. Toxic oil syndrome. A long-term follow-up of a cohort of 332 patients. Medicine (Baltimore). 1993 Sep. 72(5):285-95. [Medline].

  62. Muwakkit S, Al-Ajam M, Arayssi T, Hasbini D, Masri AF, Mikati M. Isolated digital gangrene complicating hepatitis a infection in a child. J Clin Rheumatol. 2002 Aug. 8(4):223-7. [Medline].

  63. Powell KR. Raynaud's phenomenon preceding acute lymphocytic leukemia. J Pediatr. 1973 Mar. 82(3):539-40. [Medline].

  64. Mirkinson LJ, Ratnayaka K, Sable CA, Gaskin PR. Carney complex presenting with Raynaud's phenomenon and erythematous macules of the extremities. Pediatr Cardiol. 2006 Mar-Apr. 27(2):297-300. [Medline].

  65. El-Garf AK, Mahmoud GA, Mahgoub EH. Hypermobility among Egyptian children: prevalence and features. J Rheumatol. 1998 May. 25(5):1003-5. [Medline].

  66. Haberhauer G, Feyertag J, Hanusch-Enserer U, Dunky A. Joint hypermobility syndrome and bilateral total occlusion of the ulnar arteries presenting as Raynaud's phenomenon. Clin Exp Rheumatol. 2000 Mar-Apr. 18(2):270-1. [Medline].

  67. Ervasti O, Hassi J, Rintamaki H, et al. Sequelae of moderate finger frostbite as assessed by subjective sensations, clinical signs, and thermophysiological responses. Int J Circumpolar Health. 2000 Apr. 59(2):137-45. [Medline].

  68. Metyas SK, Hoffman HM. Anakinra prevents symptoms of familial cold autoinflammatory syndrome and Raynaud's disease. J Rheumatol. 2006 Oct. 33(10):2085-7. [Medline].

  69. Monticone G, Colonna L, Palermi G, Bono R, Puddu P. Quantitative nailfold capillary microscopy findings in patients with acrocyanosis compared with patients having systemic sclerosis and control subjects. J Am Acad Dermatol. 2000 May. 42(5 Pt 1):787-90. [Medline].

  70. Allen E. and Brown G. Raynaud's disease affecting men.". Ann Intern Med. 1932. 5:1384-6.

  71. 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 Aug. 83(8):495-6. [Medline].

  72. Goette DK. Chilblains (perniosis). J Am Acad Dermatol. 1990 Aug. 23(2 Pt 1):257-62. [Medline].

  73. Crowson AN, Magro CM. Idiopathic perniosis and its mimics: a clinical and histological study of 38 cases. Hum Pathol. 1997 Apr. 28(4):478-84. [Medline].

  74. 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 May-Jun. 31(3):463-8. [Medline].

  75. Jacobs J. Pediatric Rheumatology for the Practitioner. New York: Springer-Verlag; 1993.

  76. Cook-Norris RH, Tollefson 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 Mar. 66(3):416-23. [Medline].

  77. Weiner ES, Hildebrandt S, Senecal JL, et al. Prognostic significance of anticentromere antibodies and anti-topoisomerase I antibodies in Raynaud's disease. A prospective study. Arthritis Rheum. 1991 Jan. 34(1):68-77. [Medline].

  78. Navon P, Yarom A, Davis E. Raynaud's features in childhood. Clinical, immunological and capillaroscopic study. J Mal Vasc. 1992. 17(4):273-6. [Medline].

  79. Caccavo D, Del Porto F, Garzia P, Mitterhofer AP, Galluzzo S, Rigon A. Raynaud's phenomenon and antiphospholipid antibodies in systemic lupus erythematosus: is there an association?. Ann Rheum Dis. 2003 Oct. 62(10):1003-5. [Medline].

  80. Herrick AL, Clark S. Quantifying digital vascular disease in patients with primary Raynaud's phenomenon and systemic sclerosis. Ann Rheum Dis. 1998 Feb. 57(2):70-8. [Medline].

  81. 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 Aug 15. 61(8):1103-11. [Medline].

  82. Schmidt WA, Krause A, Schicke B, Wernicke D. Color Doppler ultrasonography of hand and finger arteries to differentiate primary from secondary forms of Raynaud's phenomenon. J Rheumatol. 2008 Aug. 35(8):1591-8. [Medline].

  83. Kim SH, Kim HO, Jeong YG, Lee SY, Yoo WH, Choi TH. The diagnostic accuracy of power Doppler ultrasonography for differentiating secondary from primary Raynaud's phenomenon in undifferentiated connective tissue disease. Clin Rheumatol. 2008 Jun. 27(6):783-6. [Medline].

  84. Toprak U, Hayretci M, Erhuner Z, Tascilar K, Ates A, Karaaslan Y. Dynamic Doppler evaluation of the hand arteries to distinguish between primary and secondary raynaud phenomenon. AJR Am J Roentgenol. 2011 Jul. 197(1):W175-80. [Medline].

  85. Herron GS, Romero LI. Vascular abnormalities in scleroderma. Semin Cutan Med Surg. 1998 Mar. 17(1):12-7. [Medline].

  86. Sporbeck B, Mathiske-Schmidt K, Jahr S, Huscher D, Becker M, Riemekasten G. Effect of biofeedback and deep oscillation on Raynaud's phenomenon secondary to systemic sclerosis: results of a controlled prospective randomized clinical trial. Rheumatol Int. 2012 May. 32(5):1469-73. [Medline].

  87. Landry GJ. Current medical and surgical management of Raynaud's syndrome. J Vasc Surg. 2013 Jun. 57(6):1710-6. [Medline].

  88. Schlager O, Gschwandtner ME, Mlekusch I, Herberg K, Frohner T, Schillinger M. Auricular electroacupuncture reduces frequency and severity of Raynaud attacks. Wien Klin Wochenschr. 2011 Feb. 123(3-4):112-6. [Medline].

  89. al-Awami M, Schillinger M, Maca T, Pollanz S, Minar E. Low level laser therapy for treatment of primary and secondary Raynaud's phenomenon. Vasa. 2004 Feb. 33(1):25-9. [Medline].

  90. Herrick AL. Management of Raynaud's phenomenon and digital ischemia. Curr Rheumatol Rep. 2013 Jan. 15(1):303. [Medline].

  91. Kim YH, Ng SW, Seo HS, Chang Ahn H. Classification of Raynaud's disease based on angiographic features. J Plast Reconstr Aesthet Surg. 2011 Nov. 64(11):1503-11. [Medline].

  92. Coveliers HM, Hoexum F, Nederhoed JH, Wisselink W, Rauwerda JA. Thoracic sympathectomy for digital ischemia: a summary of evidence. J Vasc Surg. 2011 Jul. 54(1):273-7. [Medline].

  93. Bredie SJ, Jong MC. No Significant Effect of Ginkgo Biloba Special Extract EGb 761 in the Treatment of Primary Raynaud's Phenomenon: A Randomized Controlled Trial. J Cardiovasc Pharmacol. 2011 Oct 25. [Medline].

  94. Stewart M, Morling JR. Oral vasodilators for primary Raynaud's phenomenon. Cochrane Database Syst Rev. 2012. 7:CD006687. [Medline].

  95. Kowal-Bielecka O, Landewé R, Avouac J, Chwiesko S, Miniati I, Czirjak L. EULAR recommendations for the treatment of systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group (EUSTAR). Ann Rheum Dis. 2009 May. 68(5):620-8. [Medline].

  96. Walker KM, Pope J. Treatment of systemic sclerosis complications: what to use when first-line treatment fails--a consensus of systemic sclerosis experts. Semin Arthritis Rheum. 2012 Aug. 42(1):42-55. [Medline].

  97. Robertson J. and Shlikofski N. The Harriet Lane Handbook. Hospital of Johns Hopkins. Philadelphia: Elsevier; 2005.

  98. Zulian F, Corona F, Gerloni V, et al. Safety and efficacy of iloprost for the treatment of ischaemic digits in paediatric connective tissue diseases. Rheumatology (Oxford). 2004 Feb. 43(2):229-33. [Medline].

  99. Caravita S, Wu SC, Secchi MB, Dadone V, Bencini C, Pierini S. Long-term effects of intermittent Iloprost infusion on pulmonary arterial pressure in connective tissue disease. Eur J Intern Med. 2011 Oct. 22(5):518-21. [Medline].

  100. Marasini B, Massarotti M, Bottasso B, et al. Comparison between iloprost and alprostadil in the treatment of Raynaud's phenomenon. Scand J Rheumatol. 2004. 33(4):253-6. [Medline].

  101. Cooke ED, Bowcock SA, Watkins CJ, Rustin MH, Kirby JD. CL115,347, an analogue of prostaglandin E2. Peripheral circulatory effects of single ascending doses administered transdermally in normal subjects and in patients with Raynaud's phenomenon. Angiology. 1985 Dec. 36(12):867-71. [Medline].

  102. Roustit M, Blaise S, Allanore Y, Carpentier PH, Caglayan E, Cracowski JL. Phosphodiesterase-5 inhibitors for the treatment of secondary Raynaud's phenomenon: systematic review and meta-analysis of randomised trials. Ann Rheum Dis. 2013 Oct. 72(10):1696-9. [Medline].

  103. Fries R, Shariat K, von Wilmowsky H, Bohm M. Sildenafil in the treatment of Raynaud's phenomenon resistant to vasodilatory therapy. Circulation. 2005 Nov 8. 112(19):2980-5. [Medline].

  104. Gore J, Silver R. Oral sildenafil for the treatment of Raynaud's phenomenon and digital ulcers secondary to systemic sclerosis. Ann Rheum Dis. 2005 Sep. 64(9):1387. [Medline].

  105. Caglayan E, Axmann S, Hellmich M, Moinzadeh P, Rosenkranz S. Vardenafil for the treatment of raynaud phenomenon: a randomized, double-blind, placebo-controlled crossover study. Arch Intern Med. 2012 Aug 13. 172(15):1182-4. [Medline].

  106. Caglayan E, Huntgeburth M, Karasch T, Weihrauch J, Hunzelmann N, Krieg T. Phosphodiesterase type 5 inhibition is a novel therapeutic option in Raynaud disease. Arch Intern Med. 2006 Jan 23. 166(2):231-3. [Medline].

  107. Schiopu E, Hsu VM, Impens AJ, Rothman JA, McCloskey DA, Wilson JE. Randomized placebo-controlled crossover trial of tadalafil in Raynaud's phenomenon secondary to systemic sclerosis. J Rheumatol. 2009 Oct. 36(10):2264-8. [Medline].

  108. Shenoy PD, Kumar S, Jha LK, Choudhary SK, Singh U, Misra R. Efficacy of tadalafil in secondary Raynaud's phenomenon resistant to vasodilator therapy: a double-blind randomized cross-over trial. Rheumatology (Oxford). 2010 Dec. 49(12):2420-8. [Medline].

  109. Takatsuki S, Calderbank M, Ivy DD. Initial experience with tadalafil in pediatric pulmonary arterial hypertension. Pediatr Cardiol. 2012 Jun. 33(5):683-8. [Medline].

  110. Korn JH, Mayes M, Matucci Cerinic M, et al. Digital ulcers in systemic sclerosis: prevention by treatment with bosentan, an oral endothelin receptor antagonist. Arthritis Rheum. 2004 Dec. 50(12):3985-93. [Medline].

  111. Matucci-Cerinic M, Denton CP, Furst DE, Mayes MD, Hsu VM, Carpentier P. Bosentan treatment of digital ulcers related to systemic sclerosis: results from the RAPIDS-2 randomised, double-blind, placebo-controlled trial. Ann Rheum Dis. 2011 Jan. 70(1):32-8. [Medline].

  112. Barst RJ, Ivy D, Dingemanse J, et al. Pharmacokinetics, safety, and efficacy of bosentan in pediatric patients with pulmonary arterial hypertension. Clin Pharmacol Ther. 2003 Apr. 73(4):372-82. [Medline].

  113. Shimizu M, Hashida Y, Ueno K, Yokoyama T, Nakayama Y, Saito T. Successful treatment with bosentan for pulmonary hypertension and reduced peripheral circulation in juvenile systemic sclerosis. Pediatr Cardiol. 2011 Oct. 32(7):1040-2. [Medline].

  114. Denton CP, Bunce TD, Dorado MB, et al. Probucol improves symptoms and reduces lipoprotein oxidation susceptibility in patients with Raynaud's phenomenon. Rheumatology (Oxford). 1999 Apr. 38(4):309-15. [Medline].

  115. Rosato E, Borghese F, Pisarri S, Salsano F. The treatment with N-acetylcysteine of Raynaud's phenomenon and ischemic ulcers therapy in sclerodermic patients: a prospective observational study of 50 patients. Clin Rheumatol. 2009 Dec. 28(12):1379-84. [Medline].

  116. Abou-Raya A, Abou-Raya S, Helmii M. Statins: potentially useful in therapy of systemic sclerosis-related Raynaud's phenomenon and digital ulcers. J Rheumatol. 2008 Sep. 35(9):1801-8. [Medline].

  117. Fava A, Wung PK, Wigley FM, Hummers LK, Daya NR, Ghazarian SR. Efficacy of Rho kinase inhibitor fasudil in secondary Raynaud's phenomenon. Arthritis Care Res (Hoboken). 2012 Jun. 64(6):925-9. [Medline].

  118. Iorio ML, Masden DL, Higgins JP. Botulinum toxin A treatment of Raynaud's phenomenon: a review. Semin Arthritis Rheum. 2012 Feb. 41(4):599-603. [Medline].

  119. Haroon M, O'Gradaigh D, Foley-Nolan D. A case of Raynaud's phenomenon in mixed connective tissue disease responding to rituximab therapy. Rheumatology (Oxford). 2007 Apr. 46(4):718-9. [Medline].

  120. Thompson AE, Shea B, Welch V, Fenlon D, Pope JE. Calcium-channel blockers for Raynaud's phenomenon in systemic sclerosis. Arthritis Rheum. 2001 Aug. 44(8):1841-7. [Medline].

  121. Thompson AE, Pope JE. Calcium channel blockers for primary Raynaud's phenomenon: a meta-analysis. Rheumatology (Oxford). 2005 Feb. 44(2):145-50. [Medline].

  122. Herrick AL, Jayson MI. Primary Raynaud's phenomenon in early childhood. Br J Rheumatol. 1991 Jun. 30(3):223-5. [Medline].

  123. Kallenberg CG, Wouda AA, Meems L, Wesseling H. Once daily felodipine in patients with primary Raynaud's phenomenon. Eur J Clin Pharmacol. 1991. 40(3):313-5. [Medline].

  124. Wise RA, Wigley FM, White B, et al. Efficacy and tolerability of a selective alpha(2C)-adrenergic receptor blocker in recovery from cold-induced vasospasm in scleroderma patients: a single-center, double-blind, placebo-controlled, randomized crossover study. Arthritis Rheum. 2004 Dec. 50(12):3994-4001. [Medline].

  125. Herrick AL, Murray AK, Ruck A, Rouru J, Moore TL, Whiteside J, et al. A double-blind, randomized, placebo-controlled crossover trial of the a2C-adrenoceptor antagonist ORM-12741 for prevention of cold-induced vasospasm in patients with systemic sclerosis. Rheumatology (Oxford). 2014 Jan 31. [Medline].

  126. Zandman-Goddard G, Tweezer-Zaks N, Shoenfeld Y. New therapeutic strategies for systemic sclerosis--a critical analysis of the literature. Clin Dev Immunol. 2005 Sep. 12(3):165-73. [Medline].

  127. Teh LS, Manning J, Moore T, Tully MP, O'Reilly D, Jayson MI. Sustained-release transdermal glyceryl trinitrate patches as a treatment for primary and secondary Raynaud's phenomenon. Br J Rheumatol. 1995 Jul. 34(7):636-41. [Medline].

  128. Chung L, Shapiro L, Fiorentino D, Baron M, Shanahan J, Sule S. MQX-503, a novel formulation of nitroglycerin, improves the severity of Raynaud's phenomenon: a randomized, controlled trial. Arthritis Rheum. 2009 Mar. 60(3):870-7. [Medline].

  129. Spencer-Green G. Outcomes in primary Raynaud phenomenon: a meta-analysis of the frequency, rates, and predictors of transition to secondary diseases. Arch Intern Med. 1998 Mar 23. 158(6):595-600. [Medline].

  130. Hirschl M, Hirschl K, Lenz M, Katzenschlager R, Hutter HP, Kundi M. Transition from primary Raynaud's phenomenon to secondary Raynaud's phenomenon identified by diagnosis of an associated disease: results of ten years of prospective surveillance. Arthritis Rheum. 2006 Jun. 54(6):1974-81. [Medline].

  131. Navon P, Halevi A, Brand A, Branski D, Rubinow A. Progressive systemic sclerosis sine scleroderma in a child presenting as nocturnal seizures and Raynaud's phenomenon. Acta Paediatr. 1993 Jan. 82(1):122-3. [Medline].

  132. Gliddon AE, Doré CJ, Black CM, McHugh N, Moots R, Denton CP. Prevention of vascular damage in scleroderma and autoimmune Raynaud's phenomenon: a multicenter, randomized, double-blind, placebo-controlled trial of the angiotensin-converting enzyme inhibitor quinapril. Arthritis Rheum. 2007 Nov. 56(11):3837-46. [Medline].

  133. Herrick AL. Pathogenesis of Raynaud's phenomenon. Rheumatology (Oxford). 2005 May. 44(5):587-96. [Medline].

  134. Herrick AL, van den Hoogen F, Gabrielli A, Tamimi N, Reid C, O'Connell D. Modified-release sildenafil reduces Raynaud's phenomenon attack frequency in limited cutaneous systemic sclerosis. Arthritis Rheum. 2011 Mar. 63(3):775-82. [Medline].

  135. Levien TL. Phosphodiesterase inhibitors in Raynaud's phenomenon. Ann Pharmacother. 2006 Jul-Aug. 40(7-8):1388-93. [Medline].

  136. Page SM, McKenna DS. Vasospasm of the nipple presenting as painful lactation. Obstet Gynecol. 2006 Sep. 108(3 Pt 2):806-8. [Medline].

  137. Raja SG, Danton MD, MacArthur KJ, Pollock JC. Effects of escalating doses of sildenafil on hemodynamics and gas exchange in children with pulmonary hypertension and congenital cardiac defects. J Cardiothorac Vasc Anesth. 2007 Apr. 21(2):203-7. [Medline].

  138. Toprak U, Hayretci M, Erhuner Z, Tascilar K, Ates A, Karaaslan Y, et al. Dynamic Doppler evaluation of the hand arteries to distinguish between primary and secondary raynaud phenomenon. AJR Am J Roentgenol. 2011 Jul. 197(1):W175-80. [Medline].

  139. Wood HM, Ernst ME. Renin-angiotensin system mediators and Raynaud's phenomenon. Ann Pharmacother. 2006 Nov. 40(11):1998-2002. [Medline].

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Raynaud phenomenon showing demarcation of color difference.
Nine-year-old with Raynaud phenomenon. Notice discoloration of fingers.
 
 
 
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