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Physical Medicine and Rehabilitation for Complex Regional Pain Syndromes Differential Diagnoses

  • Author: Manish K Singh, MD; Chief Editor: Stephen Kishner, MD, MHA  more...
 
Updated: Mar 04, 2015
 
 

Diagnostic Considerations

Erythromelalgia is a rare and poorly understood clinical syndrome characterized by hot, red, painful extremities. The condition commonly affects the lower extremities. The application of cold or elevation of the extremity may provide pain relief.

The syndrome is classified either as primary (idiopathic) erythromelalgia or as secondary erythromelalgia, which is commonly associated with myeloproliferative, syndrome-related thrombocythemia. Adults with erythromelalgia may get relief with a daily dose of aspirin.

Differential Diagnoses

 
 
Contributor Information and Disclosures
Author

Manish K Singh, MD Assistant Professor, Department of Neurology, Teaching Faculty for Pain Management and Neurology Residency Program, Hahnemann University Hospital, Drexel College of Medicine; Medical Director, Neurology and Pain Management, Jersey Institute of Neuroscience

Manish K Singh, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pain Medicine, American Headache Society, American Association of Physicians of Indian Origin, American Medical Association, American Society of Regional Anesthesia and Pain Medicine

Disclosure: Nothing to disclose.

Coauthor(s)

Patrick M Foye, MD Director of Coccyx Pain Center, Professor and Interim Chair of Physical Medicine and Rehabilitation, Rutgers New Jersey Medical School; Co-Director of Musculoskeletal Fellowship, Co-Director of Back Pain Clinic, University Hospital

Patrick M Foye, MD is a member of the following medical societies: American Academy of Physical Medicine and Rehabilitation, International Spine Intervention Society, American Association of Neuromuscular and Electrodiagnostic Medicine, Association of Academic Physiatrists

Disclosure: Nothing to disclose.

Jashvant Patel, MBBS, MS, MD 

Jashvant Patel, MBBS, MS, MD is a member of the following medical societies: Alberta Medical Association, American Academy of Pain Medicine, American Academy of Physical Medicine and Rehabilitation, American Medical Association, American Society of Regional Anesthesia and Pain Medicine, Medical Society of the State of New York

Disclosure: Nothing to disclose.

John Grothusen, PhD Director of Quantitative Sensory and Autonomic Nervous System Laboratory, Associate Professor, Department of Neurology, Drexel University College of Medicine

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Patrick M Foye, MD Director of Coccyx Pain Center, Professor and Interim Chair of Physical Medicine and Rehabilitation, Rutgers New Jersey Medical School; Co-Director of Musculoskeletal Fellowship, Co-Director of Back Pain Clinic, University Hospital

Patrick M Foye, MD is a member of the following medical societies: American Academy of Physical Medicine and Rehabilitation, International Spine Intervention Society, American Association of Neuromuscular and Electrodiagnostic Medicine, Association of Academic Physiatrists

Disclosure: Nothing to disclose.

Chief Editor

Stephen Kishner, MD, MHA Professor of Clinical Medicine, Physical Medicine and Rehabilitation Residency Program Director, Louisiana State University School of Medicine in New Orleans

Stephen Kishner, MD, MHA is a member of the following medical societies: American Academy of Physical Medicine and Rehabilitation, American Association of Neuromuscular and Electrodiagnostic Medicine

Disclosure: Nothing to disclose.

Acknowledgements

Debra Ibrahim New York College of Osteopathic Medicine

Disclosure: Nothing to disclose.

Leia Rispoli Rutgers New Jersey Medical School

Disclosure: Nothing to disclose.

References
  1. Paice E. Reflex sympathetic dystrophy. BMJ. 1995 Jun 24. 310(6995):1645-8. [Medline]. [Full Text].

  2. Uceyler N, Eberle T, Rolke R, et al. Differential expression patterns of cytokines in complex regional pain syndrome. Pain. Sep 2007. [Medline].

  3. Wasner G, Heckmann K, Maier C, et al. Vascular abnormalities in acute reflex sympathetic dystrophy (CRPS I): complete inhibition of sympathetic nerve activity with recovery. Arch Neurol. 1999 May. 56(5):613-20. [Medline]. [Full Text].

  4. Kurvers HA, Jacobs MJ, Beuk RJ, et al. The spinal component to skin blood flow abnormalities in reflex sympathetic dystrophy. Arch Neurol. 1996 Jan. 53(1):58-65. [Medline].

  5. Blaes F, Tschernatsch M, Braeu ME, Matz O, Schmitz K, Nascimento D. Autoimmunity in complex-regional pain syndrome. Ann N Y Acad Sci. 2007 Jun. 1107:168-73. [Medline].

  6. Veldman PH, Reynen HM, Arntz IE, Goris RJ. Signs and symptoms of reflex sympathetic dystrophy: prospective study of 829 patients. Lancet. 1993 Oct 23. 342(8878):1012-6. [Medline].

  7. Maleki J, LeBel AA, Bennett GJ, et al. Patterns of spread in complex regional pain syndrome, type I (reflex sympathetic dystrophy). Pain. 2000 Dec 1. 88(3):259-66. [Medline].

  8. Peterlin BL, Rosso AL, Nair S, et al. Migraine may be a risk factor for the development of complex regional pain syndrome. Cephalalgia. 2009 Jul 9. [Medline].

  9. Werner R, Davidoff G, Jackson MD, et al. Factors affecting the sensitivity and specificity of the three-phase technetium bone scan in the diagnosis of reflex sympathetic dystrophy syndrome in the upper extremity. J Hand Surg [Am]. 1989 May. 14(3):520-3. [Medline].

  10. Kozin F, Soin JS, Ryan LM, et al. Bone scintigraphy in the reflex sympathetic dystrophy syndrome. Radiology. 1981 Feb. 138(2):437-43. [Medline]. [Full Text].

  11. Schürmann M, Zaspel J, Löhr P, et al. Imaging in early posttraumatic complex regional pain syndrome: a comparison of diagnostic methods. Clin J Pain. 2007 Jun. 23(5):449-57. [Medline].

  12. Bruehl S, Lubenow TR, Nath H, Ivankovich O. Validation of thermography in the diagnosis of reflex sympathetic dystrophy. Clin J Pain. 1996 Dec. 12(4):316-25. [Medline].

  13. Chelimsky TC, Low PA, Naessens JM, et al. Value of autonomic testing in reflex sympathetic dystrophy. Mayo Clin Proc. 1995 Nov. 70(11):1029-40. [Medline].

  14. Brunnekreef JJ, Oosterhof J, Wolff AP, et al. No impaired hemoglobin oxygenation in forearm muscles of patients with chronic CRPS-1. Clin J Pain. 2009 Jul-Aug. 25(6):513-9. [Medline].

  15. [Guideline] Harden RN, Oaklander AL, Burton AW, et al. Complex regional pain syndrome: practical diagnostic and treatment guidelines, 4th edition. Pain Med. 2013 Feb. 14(2):180-229. [Medline]. [Full Text].

  16. Rashiq S, Galer BS. Proximal myofascial dysfunction in complex regional pain syndrome: a retrospective prevalence study. Clin J Pain. 1999 Jun. 15(2):151-3. [Medline].

  17. Steverens VL, Oerlemans HM, Weesels A. Cost effectiveness analysis of adjuring physical or occupational therapy for patients with RSD. Arch Phys Med Rehabilitation. 1999. 80:1038-43.

  18. Johnston EC, Howell SJ. Tension neuropathy of the superficial peroneal nerve: associated conditions and results of release. Foot Ankle Int. 1999 Sep. 20(9):576-82. [Medline].

  19. Eckmann MS, Ramamurthy S, Griffin JG. Intravenous regional ketorolac and lidocaine in the treatment of complex regional pain syndrome of the lower extremity: a randomized, double-blinded, crossover study. Clin J Pain. 2011 Mar-Apr. 27(3):203-6. [Medline].

  20. Kumar K, Nath RK, Toth C. Spinal cord stimulation is effective in the management of reflex sympathetic dystrophy. Neurosurgery. 1997 Mar. 40(3):503-8; discussion 508-9. [Medline].

  21. Geurts JW, Smits H, Kemler MA, et al. Spinal cord stimulation for complex regional pain syndrome type I: a prospective cohort study with long-term follow-up. Neuromodulation. 2013 Nov-Dec. 16(6):523-9; discussion 529. [Medline].

  22. van Hilten BJ, van de Beek WJ, Hoff JI, et al. Intrathecal baclofen for the treatment of dystonia in patients with reflex sympathetic dystrophy. N Engl J Med. 2000 Aug 31. 343(9):625-30. [Medline]. [Full Text].

  23. Lundborg C, Dahm P, Nitescu P, et al. Clinical experience using intrathecal (IT) bupivacaine infusion in three patients with complex regional pain syndrome type I (CRPS-I). Acta Anaesthesiol Scand. 1999 Jul. 43(6):667-78. [Medline].

  24. Dielissen PW, Claassen AT, Veldman PH, Goris RJ. Amputation for reflex sympathetic dystrophy. J Bone Joint Surg Br. 1995 Mar. 77(2):270-3. [Medline]. [Full Text].

  25. Monti DA, Herring CL, Schwartzman RJ, et al. Personality assessment of patients with complex regional pain syndrome type I. Clin J Pain. 1998 Dec. 14(4):295-302. [Medline].

  26. Hassenbusch SJ, Stanton-Hicks M, Schoppa D, et al. Long-term results of peripheral nerve stimulation for reflex sympathetic dystrophy. J Neurosurg. 1996 Mar. 84(3):415-23. [Medline].

  27. Zollinger PE, Tuinebreijer WE, Breederveld RS, et al. Can vitamin C prevent complex regional pain syndrome in patients with wrist fractures? A randomized, controlled, multicenter dose-response study. J Bone Joint Surg Am. 2007 Jul. 89(7):1424-31. [Medline].

  28. Mbizvo GK, Nolan SJ, Nurmikko TJ, et al. Placebo Responses in Long-Standing Complex Regional Pain Syndrome: A Systematic Review and Meta-Analysis. J Pain. 2015 Feb. 16(2):99-115. [Medline].

  29. Hsu ES. Practical management of complex regional pain syndrome. Am J Ther. 2009 Mar-Apr. 16(2):147-54. [Medline].

  30. Quang-Cantagrel ND, Wallace MS, Magnuson SK. Opioid substitution to improve the effectiveness of chronic noncancer pain control: a chart review. Anesth Analg. 2000 Apr. 90(4):933-7. [Medline]. [Full Text].

  31. Rowbotham M, Harden N, Stacey B, et al. Gabapentin for the treatment of postherpetic neuralgia: a randomized controlled trial. JAMA. 1998 Dec 2. 280(21):1837-42. [Medline].

  32. Jain KK. An evaluation of intrathecal ziconotide for the treatment of chronic pain. Expert Opin Investig Drugs. 2000 Oct. 9(10):2403-10. [Medline].

  33. Kiefer RT, Rohr P, Ploppa A, et al. Complete recovery from intractable complex regional pain syndrome, CRPS-type I, following anesthetic ketamine and midazolam. Pain Pract. 2007 Jun. 7(2):147-50. [Medline].

  34. Koffler SP, Hampstead BM, Irani F, et al. The neurocognitive effects of 5 day anesthetic ketamine for the treatment of refractory complex regional pain syndrome. Arch Clin Neuropsychol. 2007 Aug. 22(6):719-29. [Medline].

  35. Dumitru D. Reflex sympathetic dystrophy. Phys Med Rehabil: State Art Rev. 1991. 5:89-101.

  36. Baron R. Peripheral neuropathic pain: from mechanisms to symptoms. Clin J Pain. 2000 Jun. 16(2 Suppl):S12-20. [Medline].

  37. Baron R, Maier C. Reflex sympathetic dystrophy: skin blood flow, sympathetic vasoconstrictor reflexes and pain before and after surgical sympathectomy. Pain. 1996 Oct. 67(2-3):317-26. [Medline].

  38. Bhatia KP, Bhatt MH, Marsden CD. The causalgia-dystonia syndrome. Brain. 1993 Aug. 116 ( Pt 4):843-51. [Medline].

  39. Blanchard J, Ramamurthy S, Walsh N, et al. Intravenous regional sympatholysis: a double-blind comparison of guanethidine, reserpine, and normal saline. J Pain Symptom Manage. 1990 Dec. 5(6):357-61. [Medline].

  40. Brown DL. Somatic or sympathetic block for reflex sympathetic dystrophy. Which is indicated?. Hand Clin. 1997 Aug. 13(3):485-97. [Medline].

  41. Bruehl S, Harden RN, Galer BS, et al. External validation of IASP diagnostic criteria for Complex Regional Pain Syndrome and proposed research diagnostic criteria. International Association for the Study of Pain. Pain. 1999 May. 81(1-2):147-54. [Medline].

  42. Clinchot DM, Lorch F. Sympathetic skin response in patients with reflex sympathetic dystrophy. Am J Phys Med Rehabil. 1996 Jul-Aug. 75(4):252-6.

  43. Cohen JS. Erythromelalgia: new theories and new therapies. J Am Acad Dermatol. 2000 Nov. 43(5 Pt 1):841-7. [Medline].

  44. Davis MD, O''Fallon WM, Rogers RS 3rd, Rooke TW. Natural history of erythromelalgia: presentation and outcome in 168 patients. Arch Dermatol. 2000 Mar. 136(3):330-6. [Medline]. [Full Text].

  45. Galer BS, Bruehl S, Harden RN. IASP diagnostic criteria for complex regional pain syndrome: a preliminary empirical validation study. International Association for the Study of Pain. Clin J Pain. 1998 Mar. 14(1):48-54. [Medline].

  46. Goldstein DJ, Lu Y, Detke MJ, et al. Duloxetine vs. placebo in patients with painful diabetic neuropathy. Pain. 2005 Jul. 116(1-2):109-18. [Medline].

  47. Harden RN. A clinical approach to complex regional pain syndrome. Clin J Pain. 2000 Jun. 16(2 Suppl):S26-32. [Medline].

  48. Hewitt DJ. The use of NMDA-receptor antagonists in the treatment of chronic pain. Clin J Pain. 2000 Jun. 16(2 Suppl):S73-9. [Medline].

  49. Hopp J, Schwartzman RJ, Grothusen J. Increased sensory thresholds in patients with reflex sympathetic dystrophy. Neurology. Feb 1996. 46 A:227.

  50. Hord ED, Oaklander AL. Complex regional pain syndrome: a review of evidence-supported treatment options. Curr Pain Headache Rep. 2003 Jun. 7(3):188-96. [Medline].

  51. Kemler MA, Barendse GA, van Kleef M, et al. Spinal cord stimulation in patients with chronic reflex sympathetic dystrophy. N Engl J Med. 2000 Aug 31. 343(9):618-24. [Medline]. [Full Text].

  52. Kemler MA, van de Vusse AC, van den Berg-Loonen EM, et al. HLA-DQ1 associated with reflex sympathetic dystrophy. Neurology. 1999 Oct 12. 53(6):1350-1. [Medline].

  53. Kingery WS. A critical review of controlled clinical trials for peripheral neuropathic pain and complex regional pain syndromes. Pain. 1997 Nov. 73(2):123-39. [Medline].

  54. Kurvers HA, Jacobs MJ, Beuk RJ, et al. Reflex sympathetic dystrophy: evolution of microcirculatory disturbances in time. Pain. 1995 Mar. 60(3):333-40. [Medline].

  55. Linson MA, Leffert R, Todd DP. The treatment of upper extremity reflex sympathetic dystrophy with prolonged continuous stellate ganglion blockade. J Hand Surg [Am]. 1983 Mar. 8(2):153-9. [Medline].

  56. Merskey H, Bogduk N. Classification of Chronic Pain, Description of Chronic Pain Syndrome and Definitions of Pain Terms. Seattle, Wash: IASP Press; 1994.

  57. Norton JV, Zager E, Grady JF. Erythromelalgia: diagnosis and classification. J Foot Ankle Surg. 1999 May-Jun. 38(3):238-41. [Medline].

  58. Ochoa JL. Reflex sympathetic dystrophy: a disease of medical understanding. Clin J Pain. 1992 Dec. 8(4):363-6; discussion 367-9. [Medline].

  59. Pak TJ, Martin GM, Magness JL, et al. Reflex sympathetic dystrophy. Review of 140 cases. Minn Med. 1970 May. 53(5):507-12. [Medline].

  60. Portenoy RK, Foley KM. Chronic use of opioid analgesics in non-malignant pain: report of 38 cases. Pain. 1986 May. 25(2):171-86. [Medline].

  61. Rommel O, Tegenthoff M, Pern U, et al. Sympathetic skin response in patients with reflex sympathetic dystrophy. Clin Auton Res. 1995 Sep. 5(4):205-10. [Medline].

  62. Schwartzman RJ. Explaining reflex sympathetic dystrophy. Arch Neurol. 1999 May. 56(5):521-2. [Medline].

  63. Schwartzman RJ. New treatments for reflex sympathetic dystrophy. N Engl J Med. 2000 Aug 31. 343(9):654-6. [Medline].

  64. Schwartzman RJ, Kerrigan J. The movement disorder of reflex sympathetic dystrophy. Neurology. 1990 Jan. 40(1):57-61. [Medline].

  65. Schwartzman RJ, Liu JE, Smullens SN, et al. Long-term outcome following sympathectomy for complex regional pain syndrome type 1 (RSD). J Neurol Sci. 1997 Sep 10. 150(2):149-52. [Medline].

  66. Schwartzman RJ, Maleki J. Postinjury neuropathic pain syndromes. Med Clin North Am. 1999 May. 83(3):597-626. [Medline].

  67. Stanton-Hicks M, Baron R, Boas R, et al. Complex regional pain syndromes: guidelines for therapy. Clin J Pain. 1998 Jun. 14(2):155-66. [Medline].

  68. Stanton-Hicks M, Janig W, Hassenbusch S, et al. Reflex sympathetic dystrophy: changing concepts and taxonomy. Pain. 1995 Oct. 63(1):127-33. [Medline].

  69. Tahmoush AJ, Schwartzman RJ, Hopp JL, et al. Quantitative sensory studies in complex regional pain syndrome type 1/RSD. Clin J Pain. 2000 Dec. 16(4):340-4. [Medline].

  70. van der Laan L, Veldman PH, Goris RJ. Severe complications of reflex sympathetic dystrophy: infection, ulcers, chronic edema, dystonia, and myoclonus. Arch Phys Med Rehabil. 1998 Apr. 79(4):424-9. [Medline].

  71. Wasner G, Backonja MM, Baron R. Traumatic neuralgias: complex regional pain syndromes (reflex sympathetic dystrophy and causalgia): clinical characteristics, pathophysiological mechanisms and therapy. Neurol Clin. 1998 Nov. 16(4):851-68. [Medline].

  72. Wechsler RJ, Frank ED, Halpern EH, et al. Percutaneous lumbar sympathetic plexus catheter placement for short- and long-term pain relief: CT technique and results. J Comput Assist Tomogr. 1998 Jul-Aug. 22(4):518-23. [Medline].

  73. Wilder RT, Berde CB, Wolohan M, et al. Reflex sympathetic dystrophy in children. Clinical characteristics and follow-up of seventy patients. J Bone Joint Surg Am. 1992 Jul. 74(6):910-9. [Medline].

  74. Zollinger PE, Tuinebreijer WE, Kreis RW, et al. Effect of vitamin C on frequency of reflex sympathetic dystrophy in wrist fractures: a randomised trial. Lancet. 1999 Dec 11. 354(9195):2025-8. [Medline].

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A 29-year-old woman with reflex sympathetic dystrophy in the right foot demonstrates discoloration of the skin and marked allodynia.
This photo shows the same patient as in the above image, following a right lumbar sympathetic block. Marked increase in the temperature of the right foot is noted, with more than 50% pain relief.
A 68-year-old woman with complex regional pain syndrome type II (causalgia).
A 36-year-old woman with right arm reflex sympathetic dystrophy and dystonic posture (movement disorder).
Normal laser Doppler study of the upper extremities. When the patient performs inspiratory gasp repeatedly during laser Doppler image acquisition, the transient capillary flow decreases are displayed easily and dramatically (as dark bands) in the pseudocolor image.
Laser Doppler study of the upper extremities in a patient with right hand reflex sympathetic dystrophy.
Laser Doppler study of the lower extremities in a 25-year-old woman with reflex sympathetic dystrophy in the right foot.
Algorithm for the management of chronic regional pain syndrome (CRPS). Resolution of this syndrome does not commonly occur, and the patient will need chronic pain management.
 
 
 
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