Complex regional pain syndrome
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Synopsis

Complex regional pain syndrome (CRPS) comprises two subtypes, depending on whether or not primary nerve injury is the main provocative component. Complex regional pain syndrome (CRPS) type 1, also known as reflex sympathetic dystrophy, is an uncommon syndrome of unknown etiology. It is characterized by symptoms of spontaneous and evoked neuropathic pain, with varied qualities such as burning, stabbing, and throbbing, as well as pain in response to nonpainful stimuli (allodynia) and pain out of proportion to stimulus (hyperalgesia). It is almost always accompanied by at least one of the following: tenderness, swelling, changes in skin color and temperature of a body part / extremity, abnormal sudomotor activity, limb hair changes, atrophy, or focal osteoporosis. There is a close association with sympathetic abnormalities, and this is often referred to as sympathetically maintained pain (SMP). There is usually a history of prior injury or surgery. CRPS type 1 is without clear nerve injury.
CRPS type 2, formerly called causalgia, has similar symptoms but with peripheral nerve injury as a major part of initiating the syndrome.
CRPS type 2, formerly called causalgia, has similar symptoms but with peripheral nerve injury as a major part of initiating the syndrome.
Codes
ICD10CM:
G56.40 – Causalgia of unspecified upper limb
G57.70 – Causalgia of unspecified lower limb
G90.50 – Complex regional pain syndrome I, unspecified
SNOMEDCT:
128200000 – Complex regional pain syndrome
G56.40 – Causalgia of unspecified upper limb
G57.70 – Causalgia of unspecified lower limb
G90.50 – Complex regional pain syndrome I, unspecified
SNOMEDCT:
128200000 – Complex regional pain syndrome
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
- Neuropathy
- Nerve injury
- Ischemia
- Lymphedema
- Postherpetic neuralgia
- Thalamic pain syndrome
- Erythromelalgia
- Compartment syndrome
- Peripheral vascular disease
- Raynaud phenomenon
- Deep vein thrombosis
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Management Pearls
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Therapy
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Drug Reaction Data
Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.
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References
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Last Reviewed:08/07/2018
Last Updated:09/05/2018
Last Updated:09/05/2018