Successful treatment with low-dose thalidomide in a patient with both Behcet's disease and complex regional pain syndrome type I - Case report

Thalidomide is a recognized treatment of Behcet's disease. Low-dose thalidomide seems 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 disease in a 33-year-old woman who responded well to thalidomide 50 mg 2 to 4 times per week. Her disease manifestations (severe orogenital ulceration, pseudofolliculitis, mild thrombophlebitis, positive pathergy response, and fatigue) were previously resistant to courses of prednisone, dapsone, colchicine, various types of mouthwash, and topical steroid preparations. She also gave a history of complex regional pain syndrome type 1 (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 thalidomide for her Behcet's disease, the pain in her left knee unexpectedly disappeared. There are rat experiments showing that thalidomide improves neuropathic pain, probably by selectively blocking tumor necrosis factor-alpha production in activated macrophages. We believe this is the first report of successful use of thalidomide in a human being with CRPS 1, and we therefore recommend that thalidomide should be con sidered in the treatment of CRPS 1.