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Reflex Sympathetic Dystrophy Clinical Presentation

  • Author: Don R Revis, Jr, MD; Chief Editor: Herbert S Diamond, MD  more...
 
Updated: Oct 16, 2015
 

History

The three clinical stages of reflex sympathetic dystrophy (RSD) are acute, subacute, and chronic. The acute form lasts approximately 3 months. Pain, often burning in nature, is one of the first symptoms that initially limits function. Swelling, redness with vasomotor instability that worsens with dependency, hyperhidrosis, and coolness to the touch are common physical findings. Demineralization of the underlying bony skeleton begins because of disuse.

If the process is not arrested or reversed in the acute phase, the condition may progress to the subacute stage, which can last for up to 9 months. The patient develops persistent severe pain in the extremity and fixed edema that would have been reversible with elevation during the acute phase. The redness of the acute stage gives way to cyanosis or pallor and hyperhidrosis to dry skin. Loss of function progresses, both because of increased pain and fibrosis of the joints caused by chronic inflammation. In the hand, this leads to flexion deformity of the fingers. The skin and subcutaneous tissues begin to atrophy. Demineralization of the underlying bony skeleton becomes pronounced.

If the process continues, the chronic phase may develop approximately 1 year after disease onset. This stage may last for many years or can be permanent. Pain is more variable during this period. It may continue undiminished or abate. Edema tends to subside over time, leaving fibrosis around the involved joints. The skin is dry, pale, cool, and shiny. Flexion and extension creases are absent. Loss of function and stiffness are marked, and osteoporosis is extreme. In the upper extremity, this can manifest as a frozen shoulder and claw hand.

A thorough general history is strongly suggested. Maintaining a high index of suspicion is important because proper treatment requires rapid diagnosis and prompt therapy.

RSD commonly involves only one extremity. It is bilateral in approximately 25% of cases, but in those cases it is usually more prominent on one side.

Pain in RSD has the following characteristics:

  • Usually constant and disproportionate to the precipitant injury
  • May be exacerbated by ambient factors such as loud noises and emotional factors (eg, stress, light touch, active motion, passive motion)
  • May be described as burning, cutting, searing, pressure, or tearing
  • Usually begins locally but may progress to involve the entire extremity

Possible evidence of prior increased sympathetic activity includes the following:

  • Hyperhidrosis
  • Cold hands
  • Fainting

Precipitating factors may include any of the following:

  • Prior trauma, which may be trivial (eg, venipuncture) or significant (eg, Colles fracture), with or without diagnosable nerve injury
  • Prior surgery
  • Recent limb immobilization due to hemiplegic stroke, myocardial infarction
  • Systemic disease such as diabetes
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Physical Examination

Perform a thorough physical examination followed by a focused examination of the involved extremity. Patients with RSD may present with suggestive physical findings that point to a presumptive diagnosis (eg, edema, stiffness, discoloration, abnormal skin moisture, tenderness).

Edema is the most consistent physical finding and is always disproportionate to the severity of the precipitant injury or event. Pain, swelling, and color change may be more prominent with dependency in the early stages. Edema worsens rather than improves and extends beyond the region of initial concern. It evolves into a brawny, nonpitting edema that may progress to an intense fibrosis in all the joints of the extremity.

Stiffness is more severe than expected and may be very distressing to the patient.

Discoloration varies depending on the stage of disease. It may be dusky, cyanotic, pale, or red and may eventually lead to skin hypopigmentation. In the hand, discoloration begins as redness over the metacarpophalangeal (MCP) or proximal interphalangeal (PIP) joint flexion creases early in the disease and progresses as a streak across the palm.

Early in the course of RSD, abnormal skin moisture consists of hyperhidrosis. Late in the course, it consists of dry skin.

Tenderness is initially localized but may progress to generalized tenderness. Exquisite tenderness, both periarticular and interarticular, is often present. Patients may exhibit allodynia (ie, pain with nonnoxious stimuli) and hyperpathia (ie, persistent pain after light pressure).

Other physical findings may include the following:

  • Atrophy of the skin and subcutaneous fat pads
  • Fibrosis of the palmar fascia
  • Absence of extensor and flexor creases over joints
  • Frozen shoulder, flexion deformities of the fingers, claw hand
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Contributor Information and Disclosures
Author

Don R Revis, Jr, MD Consulting Staff, Department of Surgery, Division of Plastic and Reconstructive Surgery, University of Florida College of Medicine

Don R Revis, Jr, MD is a member of the following medical societies: American College of Surgeons, American Society for Aesthetic Plastic Surgery, American Society of Plastic Surgeons, American Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Elliot Goldberg, MD Dean of the Western Pennsylvania Clinical Campus, Professor, Department of Medicine, Temple University School of Medicine

Elliot Goldberg, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology

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.

Lawrence H Brent, MD Associate Professor of Medicine, Jefferson Medical College of Thomas Jefferson University; Chair, Program Director, Department of Medicine, Division of Rheumatology, Albert Einstein Medical Center

Lawrence H Brent, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Physicians, American College of Rheumatology

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Janssen<br/>Serve(d) as a speaker or a member of a speakers bureau for: Abbvie; Genentech; Pfizer; Questcor.

Chief Editor

Herbert S Diamond, MD Visiting Professor of Medicine, Division of Rheumatology, State University of New York Downstate Medical Center; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, Phi Beta Kappa

Disclosure: Nothing to disclose.

Additional Contributors

Robert E Wolf, MD, PhD Professor Emeritus, Department of Medicine, Louisiana State University School of Medicine in Shreveport; Chief, Rheumatology Section, Medical Service, Overton Brooks Veterans Affairs Medical Center

Robert E Wolf, MD, PhD is a member of the following medical societies: American College of Rheumatology, Arthritis Foundation, Society for Leukocyte Biology

Disclosure: Nothing to disclose.

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

  2. 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].

  3. Sebastin SJ. Complex regional pain syndrome. Indian J Plast Surg. 2011 May. 44(2):298-307. [Medline]. [Full Text].

  4. Coderre TJ, Bennett GJ. A hypothesis for the cause of complex regional pain syndrome-type I (reflex sympathetic dystrophy): pain due to deep-tissue microvascular pathology. Pain Med. 2010 Aug. 11(8):1224-38. [Medline].

  5. Pleger B, Draganski B, Schwenkreis P, Lenz M, Nicolas V, Maier C, et al. Complex regional pain syndrome type I affects brain structure in prefrontal and motor cortex. PLoS One. 2014. 9(1):e85372. [Medline]. [Full Text].

  6. Barad MJ, Ueno T, Younger J, Chatterjee N, Mackey S. Complex regional pain syndrome is associated with structural abnormalities in pain-related regions of the human brain. J Pain. 2014 Feb. 15(2):197-203. [Medline].

  7. Lee DH, Lee KJ, Cho KI, Noh EC, Jang JH, Kim YC, et al. Brain alterations and neurocognitive dysfunction in patients with complex regional pain syndrome. J Pain. 2015 Jun. 16 (6):580-6. [Medline].

  8. Goebel A. Complex regional pain syndrome in adults. Rheumatology (Oxford). 2011 Oct. 50(10):1739-50. [Medline].

  9. Introduction and Diagnostic Considerations. Harden RN. Complex Regional Pain Syndrome: Treatment Guidelines. Milford, CT: Reflex Sympathetic Dystrophy Association; 2006. 1-11. [Full Text].

  10. O'Connell NE, Wand BM, McAuley J, Marston L, Moseley GL. Interventions for treating pain and disability in adults with complex regional pain syndrome. Cochrane Database Syst Rev. 2013 Apr 30. 4:CD009416. [Medline].

  11. 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].

  12. Gobelet C, Waldburger M, Meier JL. The effect of adding calcitonin to physical treatment on reflex sympathetic dystrophy. Pain. 1992 Feb. 48(2):171-5. [Medline].

  13. Littlejohn G. Therapy: Bisphosphonates for early complex regional pain syndrome. Nat Rev Rheumatol. 2013 Apr. 9(4):199-200. [Medline].

  14. Varenna M, Adami S, Rossini M, Gatti D, Idolazzi L, Zucchi F, et al. Treatment of complex regional pain syndrome type I with neridronate: a randomized, double-blind, placebo-controlled study. Rheumatology (Oxford). 2013 Mar. 52(3):534-42. [Medline].

  15. Taylor RS, Van Buyten JP, Buchser E. Spinal cord stimulation for complex regional pain syndrome: a systematic review of the clinical and cost-effectiveness literature and assessment of prognostic factors. Eur J Pain. 2006 Feb. 10(2):91-101. [Medline].

  16. 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].

  17. [Guideline] National Institute for Health and Care Excellence. Spinal cord stimulation for chronic pain of neuropathic or ischaemic origin. NICE. Available at http://www.nice.org.uk/guidance/ta159. Accessed: September 26, 2014.

  18. Cimaz R, Matucci-Cerinic M, Zulian F, Falcini F. Reflex sympathetic dystrophy in children. J Child Neurol. 1999 Jun. 14(6):363-7. [Medline].

  19. Badri T, Ben Jennet S, Fenniche S, Benmously R, Mokhtar I, Hammami H. Reflex sympathetic dystrophy syndrome in a child. Acta Dermatovenerol Alp Panonica Adriat. 2011 Jun. 20(2):77-9. [Medline].

  20. Bean DJ, Johnson MH, Heiss-Dunlop W, Lee AC, Kydd RR. Do psychological factors influence recovery from Complex Regional Pain Syndrome Type-1? A Prospective Study. Pain. 2015 Jul 1. [Medline].

  21. Stanton TR, Wand BM, Carr DB, Birklein F, Wasner GL, O'Connell NE. Local anaesthetic sympathetic blockade for complex regional pain syndrome. Cochrane Database Syst Rev. 2013 Aug 19. 8:CD004598. [Medline].

  22. Azari P, Lindsay DR, Briones D, Clarke C, Buchheit T, Pyati S. Efficacy and safety of ketamine in patients with complex regional pain syndrome: a systematic review. CNS Drugs. 2012 Mar 1. 26(3):215-28. [Medline].

 
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