Clinical notePatients with Ehlers Danlos syndrome and CRPS: A possible association?Stoler, Joan M.a,*; Oaklander, Anne Louiseb,c,dAuthor Information aGenetics and Teratology Unit, Department of Pediatrics, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA bNerve Injury Unit, Department of Anesthesia and Critical Care, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA cNerve Injury Unit, Department of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA dNerve Injury Unit, Department of Neuropathology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA *Corresponding author. Tel.: +1 617 726 1742; fax: +1 617 724 1911. E-mail address:[email protected] Received September 1, 2005; received in revised form February 6, 2006; accepted February 7, 2006. Pain: July 2006 - Volume 123 - Issue 1 - p 204-209 doi: 10.1016/j.pain.2006.02.022 Buy Metrics Abstract Rare patients are left with chronic pain, vasodysregulation, and other symptoms that define complex regional pain syndrome (CRPS), after limb traumas. The predisposing factors are unknown. Genetic factors undoubtedly contribute, but have not yet been identified. We report four CRPS patients also diagnosed with the classical or hypermobility forms of Ehlers Danlos syndrome (EDS), inherited disorders of connective tissue. These patients had been diagnosed using standard diagnostic criteria for CRPS and for EDS. All had sustained joint injury; in three this had been surgically treated. The association of these two diagnoses leads us to hypothesize that EDS might contribute to the development of CRPS in one or more of the following ways: via stretch injury to nerves traversing hypermobile joints, increased fragility of nerve connective tissue, or nerve trauma from more frequent surgery. We review the clinical presentation of the different Ehlers Danlos syndromes and provide clinical criteria that can be used to screen CRPS patients for EDS for clinical or research purposes. © 2006 Lippincott Williams & Wilkins, Inc.