SIGNS AND SYMPTOMS OF RSD./CRPS; diagnosis and how bad it can get-Part I

Diagnosis

If one can demonstrate major nerve damage associated with the development of RSD / CRPS symptoms, the condition is called complex regional pain syndrome (CRPS) type II or causalgia. Generally, causalgia provides more objective evidence of disease due to neurological changes (numbness and weakness).

The terms complex regional pain syndrome (CRPS) type I and type II have been used since 1995, when the International Association for the Study of Pain (IASP) felt the respective names reflex sympathetic dystrophy and causalgia were inadequate to represent the full spectrum of signs and symptoms. The term "Complex" was added to convey the reality that RSD and causalgia express varied signs and symptoms. Many publications, particularly older ones, still use the names RSD and causalgia. To facilitate communication and understanding the designation RSD / CRPS is generally used throughout these practice guidelines. The principles applicable to the diagnosis and management of RSD are similar to those principles applicable to the diagnosis and management of causalgia.

To make the early diagnosis of RSD / CRPS, the practitioner must recognize that some features/manifestations of RSD / CRPS are more characteristic of the syndrome than others, and that the clinical diagnosis is established by piecing each bit of the puzzle together until a clear picture of the disorder emerges. Often the physician needs to rule out other potentially life-threatening disorders that may have clinical features similar to RSD / CRPS, e.g. a blood clot in a leg vein or a breast tumor spreading to lymph glands can cause a swollen, painful extremity. Indeed, RSD / CRPS may be a component part of another disease, (e.g. a herniated disc of the spine, carpal tunnel syndrome of the hand, heart attack). Thus, treating RSD / CRPS will often be directed to treating clinical features rather than a well defined disease. When RSD / CRPS spreads the diagnosis can be more complicated. For example, if it spreads to the opposite limb, it may be more difficult to establish a diagnosis because there is no normal side (control) to compare for objective findings. On the other hand, the spreading of RSD / CRPS symptoms may actually facilitate the diagnosis of RSD / CRPS because spreading symptoms is a characteristic of the disorder. (See below).

Importance of Objective Findings

Many patients who develop RSD / CRPS as the result of an injury do so in the context of legal liability. Some patients can be expected to defend their rights in courts of law. It is not uncommon for the defendant to accuse the patient of faking their condition, especially if there are no objective findings for RSD / CRPS documented on the medical record. Therefore, the evaluating physician must assess more than just subjective complaints (medical history). The physician must aggressively seek and document objective findings. For example, about 80% of RSD / CRPS cases have differences in temperature in opposite sides that may be either colder or warmer. These temperature changes may be associated with changes in skin color. Furthermore, the temperature differences are not static. The skin temperature can undergo dynamic changes in a relatively short period of time (within minutes) depending critically on room temperature, local temperature of the skin and emotional stress. In some cases, the differences in temperatures may fluctuate spontaneously even without any apparent provocation. ⁴ Thus, the objective finding of differences in temperature and color of the skin can be missed by the physician if only a single physical examination is made. A useful and relatively inexpensive instrument to have available at the time of the physical examination is a portable infrared thermometer to measure differences in skin temperature. Changes in skin temperature and color are only two examples of several objective findings that should be sought in the patients with RSD / CRPS.

Making the Diagnosis of RSD / CRPS

The diagnosis of RSD / CRPS can be made in the following context. A history of trauma to the affected area associated with pain that is disproportionate to the inciting event plus evidence at some time for one or more of the following:

• Abnormal function of the sympathetic nervous system, e.g., abnormal changes in skin blood flow, sweating or goose flesh.
• Swelling.
• Movement disorder.
• Changes in tissue growth (dystrophy and atrophy).

Thus patients do not have to meet all of the clinical manifestations listed above to make the diagnosis of RSD / CRPS. Note also that the criteria state "evidence at some time" for the clinical findings. This does not mean that the evidence for the clinical finding listed has to be constant. This explains why swelling, abnormal sweating, abnormal skin temperatures, etc., are not reported by the physician on some visits. Sometimes weather plays a factor or emotional stress or the patient may be in a flare-up or a remission stage. Patients have good and bad days.

 

The RSD / CRPS diagnosis is precluded by the existence of known pathology that can be explained by the observed symptoms and degree of pain. The pain and symptoms of RSD / CRPS may exceed both the magnitude and duration of symptoms expected from the normal healing process anticipated from the inciting event. There seems to be a small group of patients whose pain following trauma resolves over time, leaving the patient with more of a movement than a pain disorder.

 

Clinical Features of RSD / CRPS

1. Pain - The hallmark of RSD / CRPS is pain and mobility problems out of proportion to those expected from the initial injury. The first and primary complaint occurring in one or more extremities is described as severe, constant, burning and/or deep aching pain. All tactile stimulation of the skin (e.g. wearing clothing, a light breeze) may be perceived as painful (allodynia). Repetitive tactile stimulation (e.g. tapping on the skin) may cause increasing pain with each tap and when the repetitive stimulation stops, there may be a prolonged after-sensation of pain (hyperpathia). There may be diffuse tenderness or point-tender spots in the muscles of the affected region due to small muscle spasms called muscle trigger points (myofascial pain syndrome). There may be spontaneous sharp jabs of pain in the affected region that seem to come from nowhere (paroxysmal dysesthesias and lancinating pains).
   
   
2. Skin changes - skin may appear shiny (dystrophy-atrophy), dry or scaly. Hair may initially grow coarse and then thin. Nails in the affected extremity may be more brittle, grow faster and then slower. Faster growing nails is almost proof that the patient has RSD / CRPS. RSD / CRPS is associated with a variety of skin disorders including rashes, ulcers and pustules. ⁹ Although extremely rare, some patients have required amputation of an extremity due to life-threatening reoccurring infections of the skin. Abnormal sympathetic (vasomotor changes) activity may be associated with skin that is either warm or cold to touch. The patient may perceive sensations of warmth or coolness in the affected limb without even touching it (vasomotor changes). The skin may show increased sweating (sudomotor changes) or increased chilling of the skin with goose flesh (pilomotor changes). Changes in skin color can range from a white mottled appearance to a red or blue appearance. Changes in skin color (and pain) can be triggered by changes in the room temperature, especially cold environments. However, many of these changes occur without any apparent provocation. Patients describe their disease as though it had a mind of its own.
   
   
   
   Photo Gallery of Skin Lesions Associated with RSD / CRPS:   Dr. Robert J. Schwartzman, whose name is synonymous with RSD / CRPS, has contributed a photo gallery to the Clinical Practice Guidelines. His photos illustrate some of the objective findings that may be observed in patients with RSD / CRPS. It should be emphasized that patients with RSD / CRPS may not present with these objective findings, especially during the early stages of the disease.
   
   Dr. Schwartzman is Professor and Chairman of the Department of Neurology at Hahnemann School of Medicine in Philadelphia, PA, USA. He is a member the Scientific Advisory Committee. Through his numerous publications, lectures, and research efforts, he shares his clinical experience by making others aware of the effects of RSD / CRPS. Dr. Schwartzman has received several honors and awards, including the Mayo Clinic Neurology Teaching Award and the Dean's Special Award for Excellence in Teaching from Hahnemann University of Medicine in both 1998 and 1999

 RSD PHOTO GALLERY, PER RSD FOUNDATION:

Anchoring fibril deficiency. The patient sheds all of the skin from her arms and upper body. Stage III of RSD

Atrophic reddened skin. Shiny skin that later desquamated. Stage III of RSD

Atrophic skin of stage III patient Combination of loss of hair and increased thickness of hair

Demonstrates brawny edema Reddened thickened skin Increased nail growth of the great toe Deep ulcerated lesion on the top of the foot. Stage I of RSD

Example of atrophic skin with deep ulcer on the top of the right foot.  Atrophic skin, loss of hair on the affected, or unaffected limb

Example of Gardner-Diamond Syndrome

Commonly seen small punctate skin lesions that will ulcerate Stage III of RSD

Example of deep ulcer stage III.

Ligature signs, bilaterally below the knees Nothing had been placed on the legs  Stage III of RSD

Example of early lesions that become ulcerated in latter stages. Stage I of RSD-yeah, doesn't itch either

 How BAD CAN it really get???

See the next post.