Successful Treatment With Low-dose Thalidomide in a Patient With Both Behçet's Disease and Complex Regional Pain Syndrome Type I: Case Report

Thalidomideis a recognized treatment of Behcet’s disease. Low-dose thalidomideseems to be effective in the treatment of orogenital ulcers and is potentially safer with a lower incidence of adverse effects than higher doses. We wish to report a case of Behcet’s diseasein a 33-year-old woman who responded well to thalidomide50 mg 2 to 4 times per week. Her disease manifestations (severe orogenital ulceration, pseudofolliculitis, mild thrombophlebitis, positive pathergyresponse, and fatigue) were previously resistant to courses of prednisone, dapsone, colchicine, various types of mouthwash, and topical steroidpreparations. She also gave a history of complex regional pain syndrometype I (CRPS 1) over her left patella (severe pain, intermittent edema, hyperalgesia, allodynia, cold skin, and loss of movement) after a fall onto her left knee 6 years previously. This had only partially responded to a variety of treatment modalities. After starting thalidomidefor her Behcet’s disease, the pain in her left knee unexpectedly disappeared. There are rat experiments showing that thalidomideimproves neuropathic pain, probably by selectively blocking tumor necrosis factor-alpha productionin activated macrophages. We believe this is the first report of successful use of thalidomidein a human being with CRPS 1, and we therefore recommend that thalidomideshould be considered in the treatment of CRPS 1.