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Complex regional pain syndrome type I (CRPS I) is a multifactorial painful disorder with a complex pathogenesis. Both peripheral and central mechanisms are involved. Acute CRPS I is considered to be an exaggerated inflammatory disorder; however, over time, because of altered function of the sympathetic nervous system and maladaptive neuroplasticity, CRPS I evolves into a neurological disorder. This review thoroughly describes the pathophysiological aspects of CRPS I and summarizes the potential therapeutic options. The mechanisms and targets of the treatment are different in the early and late stages of the disease. This current review builds on a previous review by this author group by deepening the role of the peripheral classic and neuronal inflammatory component in the acute stage of this painful disorder. Level of Evidence Not applicable.

1 O.U. of Anesthesia, Intensive Care and Pain Therapy of Sant’Antonio Abate Hospital, Erice Casa Santa, Trapani, Italy. 2 O.U. of Anesthesia, Intensive Care and Pain Therapy of Azienda Ospedaliera Ospedali Riuniti Villa Sofia Cervello, Palermo, Italy. 3 Anesthesiology, Intensive Care and Pain Therapy Department, Catholic University, Medical School, Rome, Italy. 4 Plastic and Reconstructive Surgery Department, University 'Sapienza' of Rome, Italy.

Background Complex regional pain syndrome type I (CRPS I), formerly known as reflex sympathetic dystrophy (RSD), is a chronic painful disorder that usually develops after a minor injury to a limb. This topical review gives a synopsis of CRPS I and discusses the current concepts of our understanding of CRPS I in adults, the diagnosis, and treatment options based on the limited evidence found in medical literature. CRPS I is a multifactorial disorder. Possible pathophysiological mechanisms of CRPS I are classic and neurogenic inflammation, and maladaptive neuroplasticity. At the level of the central nervous system, it has been suggested that an increased input from peripheral nociceptors alters the central processing mechanisms. Methods A literature search was conducted using, as electronic bibliographic database, Medline from 1980 until 2014. Results An early diagnosis and multidisciplinary treatment are necessary to prevent permanent disability. Conclusions The pharmacological treatment of CRPS I is empirical and insufficiently effective. Further research is needed regarding the therapeutic modalities discussed in the guidelines. Physical therapy is widely recommended as a first-line treatment. The efficacy of local anesthetic sympathetic blockade as treatment for CRPS I is questionable.