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Reflex Sympathetic Dystrophy, RSD, Complex Regional Pain Syndrome, Edema, Lymphedema, Causalgia, CRPS, Sudeck's Dystrophy, Post Traumaatic Dystrophy, Shoulder Hand Syndrome, Reflex Neurovascular Dystrophy
Reflex Sympathetic Dystrophy or Complex Regional Pain Syndrome is a chronic and debilitating condition that causes excruciating burning pain changes in bone and skin tissue, excessive sweating, sensitivity to touch and swelling (edema). The condition affects and involves the sympathetic nerve system. The causes are many and include trauma from an injury, and can also be caused by heart attacks and surgery. Other causes may involve lacerations or wounds, degenerative disc disease, burns or compression injuries, cerebral lesions, repetitive motion disorders such as carpal tunnel syndrome. This is also a very devestating condition for the patient to deal with as it is so terribly misunderstood. Psychological difficulties include and arise from lack of sleep due to the pain involved, depression in attempting to cope with the increasing disability and relationship difficulties from family and friends who don't understand either the condition or what the patient is experiencing.
Severe burning pain that is more severe than the original causative factor. A chronic burning pain in a localized area, sensitivity to temperature and touch, and a color change or discoloration of the skin. Other symptoms may include changes in nail and hair growth, joint stiffness and swelling, motor disabilities.
Stage one is thought to last from 1 to 3 months and is characterized by severe, burning pain, along with muscle spasm, joint stiffness, rapid hair growth, and alterations in the blood vessels that cause the skin to change color and temperature.
Stage two lasts from 3 to 6 months and is characterized by intensifying pain, swelling, decreased hair growth, cracked, brittle, grooved, or spotty nails, softened bones, stiff joints, and weak muscle tone. Stage two may also include a peripheral nerve lesion.
In stage three the syndrome progresses to the point where changes in the skin and bone are no longer reversible. Pain becomes unyielding and may involve the entire limb or affected area. There may be marked muscle loss (atrophy), severely limited mobility, and involuntary contractions of the muscles and tendons that flex the joints. Limbs may become contorted. (1)
Symptoms can include:
other symptoms that have been noted are:
Changes in skin temperature, color and texture. At times your skin may be sweaty; at other times it may be cold. Skin color can range from white and mottled to red or blue. Skin may become tender, thin or shiny in the affected area.
CRPS Type I (also referred to as RSD) - cases in which the nerve injury cannot be immediately identified.
CRPS Type II (also referred to as Causalgia) - cases in which a distinct “major” nerve injury has occurred.
CRPS is best described in terms of an injury to a nerve or soft tissue (e.g. broken bone) that does not follow the normal healing path.
CRPS development does not appear to depend on the magnitude of the injury. The sympathetic nervous system seems to assume an abnormal function after an injury.
Since there is no single laboratory test to diagnose CRPS, the physician must assess and document both subjective complaints (medical history) and, if present, objective findings (physical examination).(1)
There is no specific diagnostic test for RSD. Patient evaluation often focuses on the elimination of other conditions. It is also extremely difficult to diagnose because the condition varies from person to person and may even go into spontaneous remission. However, there are tests available that can assist in diagnoses. These include a thermogram, a three phase radionuclide bone scan, sympathetic nerve blocks.
Other radiological tests may be used to identify possible causes of the patients pain, although they are of limited or no value in the actual diagnosing of the condition.
The prognoses is as difficult as diagnosis because again, the condition varies so greatly from patient to patient.
Since there is no cure for the condition, treatment modalities will focus on the symptoms and complications.
Treatment modalities will include physical therapy for the affected limbs, pain management, medications for inflammatory responses, decongestive therapy for the edema, psychological counseling due to the emotional trauma of increasing disability and pain, possible sympathatic nerve blocks to alleviate pain.
If lymphedema is present and there is damage to the lymphatics, a treatment program for lymphedema management should be initiated. The lymphedema treatment program would include: Manual lymphatic drainage; compression wraps or compression bandages (using short stretch bandages), compression garments, compression sleeves.
June 13, 2008
Maknig a correction diagnosis between RSD and lymphedema can be difficult as there are such similarities in the symptoms, especially swelling.
The major difference is that RSD is considered a neurological disorder while lymphedema is a disorder of the lymphatic system.
The pain involved with RSD is described as a severe burning pain and the affected arm or leg is extremely painful to touch.
The pain involving lymphedema expresses in several ways, depending on the stage of the LE. The sub-clinical stage or in the very early stage one, where there may be transient LE, patients often describe a “needles and pins” discomfort. They also describe an “achiness” of the limb itself.
As lymphedema progresses, the pain can become an all over (sometimes brutal) achy type pain, similar to the flu.
There can also be severe pain of the limb itself and specific pain in the joints due to the wear and tear of the weight of the swollen limb.
Other complications of lymphedema that are not involved with RSD include tissue hardening (fibrosis), susceptibility towards lymphatic cancers, various skin conditions suc as dry skin, splitting, plaques and nodules, PapillomatosisPlaques including “cobblestone” appearing plaque, Dermatofibroma Skin tags Warts and Verrucas Mycetoma Skin Fungus and dermatitis. See Complications of Lymphedema for a more comprehensive description.
The symptoms of each condition are different as well. Basic symptoms for lymphedema include: Swelling in the arms, hands, fingers, shoulders, chest, or legs. The swelling may occur for the first time after a traumatic event (such as bruises, cuts, sunburn, and sports injuries), after an infection in the part of the body that was treated for cancer, or after an extended (more than three hours) airplane trip (due to the sudden change in cabin pressure).
At the present time there is no clinical evidence that lymphedema itself can or does cause RSD.
If any swelling (edema) becomes permanent and the lymph system becomes damaged, then the swelling is clinically considered to be lymphedema. So the answer is yes, RSD can cause lymphedema.
Interestingly, there is also a clinical abstract wherein the researchers found lymphatic distortions in a significant number of the tested RSD patients. Another study indicates decreased lymph flow or decrease peripheral lymph flow in RSD. (3) This was caused by an increased sympathetic stimulus to the lymphatics, possibly therefore overwhelming the lymph system’s ability to move the fluid.
NINDS Reflex Sympathetic Dystrophy Syndrome Information Page
Complex regional pain syndrome (CRPS) is a chronic pain condition. The key symptom of CRPS is continuous, intense pain out of proportion to the severity of the injury, which gets worse rather than better over time. CRPS most often affects one of the arms, legs, hands, or feet. Often the pain spreads to include the entire arm or leg. Typical features include dramatic changes in the color and temperature of the skin over the affected limb or body part, accompanied by intense burning pain, skin sensitivity, sweating, and swelling. Doctors aren’t sure what causes CRPS. In some cases the sympathetic nervous system plays an important role in sustaining the pain. Another theory is that CRPS is caused by a triggering of the immune response, which leads to the characteristic inflammatory symptoms of redness, warmth, and swelling in the affected area.
Because there is no cure for CRPS, treatment is aimed at relieving painful symptoms. Doctors may prescribe topical analgesics, antidepressants, corticosteroids, and opioids to relieve pain. However, no single drug or combination of drugs has produced consistent long-lasting improvement in symptoms. Other treatments may include physical therapy, sympathetic nerve block, spinal cord stimulation, and intrathecal drug pumps to deliver opioids and local anesthetic agents via the spinal cord.
The prognosis for CRPS varies from person to person. Spontaneous remission from symptoms occurs in certain individuals. Others can have unremitting pain and crippling, irreversible changes in spite of treatment. What research is being done?
The National Institute of Neurological Disorders and Stroke (NINDS) and other institutes of the National Institutes of Health (NIH) conduct research relating to CRPS in laboratories at the NIH and also support additional research through grants to major medical institutions across the country. NINDS-supported scientists are studying new approaches to treat CRPS and intervene more aggressively after traumatic injury to lower the chances of developing the disorder.
Reflex Sympathetic Dystrophy Syndrome Fact Sheet
Complex Regional Pain Syndrome (also called Causalgia and Reflex Sympathetic Dystrophy Syndrome) information page compiled by the National Institute of Neurological Disorders and Stroke (NINDS).
Pain: Hope Through Research
Chronic pain information page compiled by the National Institute of Neurological Disorders and Stroke (NINDS).
Reflex Sympathetic Dystrophy/ Complex Regional Pain Syndromes (CRPS): State-of-the-Science
A workshop on Reflex Sympathetic Dystrophy/ Complex Regional Pain Syndromes (CRPS): State-of-the-Science, December 15, 2001
Severe complications of reflex sympathetic dystrophy: infection, ulcers, chronic edema, dystonia, and myoclonus.
van der Laan L, Veldman PH, Goris RJ.
Department of Surgery, University Hospital Nijmegen, The Netherlands.
To determine the prevalence, type of complication, predisposing factors, and treatment for severe complications in a population of reflex sympathetic dystrophy (RSD) patients.
DESIGN: Retrospective analysis of the data from RSD patients collected over a 12-year period, to investigate the involvement of predisposing factors in an RSD population without severe complications compared with an RSD population with severe complications.
SETTING: Outpatient clinic of a department of surgery of a university hospital.
PATIENTS: A total of 1,006 patients with the diagnosis of RSD established according to prospectively defined criteria.
MAIN OUTCOME MEASURES: The signs and symptoms of every RSD patient who visited the department were prospectively documented in the medical history; these data were retrospectively analyzed with special regard to RSD with severe complications-infection, ulcers, chronic edema, dystonia, and/or myoclonus-for prevalence, type of complication, and treatment.
RESULTS: Seventy-four RSD patients who were mostly young and female developed severe complications. More than one complication occurred in 91% of the affected extremities. Severe complications developed more frequently in the lower extremity (65%). In patients in whom the acute RSD started with a decreased skin temperature of the affected extremity, severe complications developed significantly more often than in acute RSD patients with a warm skin temperature of the extremity from the onset of the disease (p < .001).
CONCLUSIONS: It is important to recognize “cold” RSD immediately at the onset of the disease because this group of RSD patients has a higher risk of developing a severe complication, mostly followed by a severe disability that is resistant to therapy.
PMID: 9552109 PubMed - indexed for MEDLINE
Online RSD support group Yahoo
Reflex Sympathetic Dystrophy eMedicine
Algoneurodystrophy Shoulder-hand syndrome Sudeck's atrophy Sympathetic reflex dystrophy
2008 ICD-9-CM Diagnosis 337.21
Reflex sympathetic dystrophy of the upper limb
2008 ICD-9-CM Diagnosis 337.22
Reflex sympathetic dystrophy of the lower limb
2008 ICD-9-CM Diagnosis 354.4
Causalgia of upper limb
2008 ICD-9-CM Diagnosis 355.71
Causalgia of lower limb
355.71 is a specific code that can be used to specify a diagnosis
355.71 contains 2 index entries