Complex regional pain syndrome (CRPS) is a chronic neuropathic pain disorder characterized by neuropathic pain associated with local edema and changes suggestive of autonomic involvement such as altered sweating, skin color, and skin temperature of the affected region. CRPS was described associated with several diseases, such as trauma, psychiatric conditions, and cancer. However, no case associated with Still's disease has been previously described. In this paper, the authors describe the first case of CRPS associated with Still's disease. 1. Introduction Complex regional pain syndrome (CRPS), also known as reflex sympathetic dystrophy and causalgia, algodystrophy, Sudeck’s atrophy, hand-shoulder syndrome, neuroalgodystrophy, and posttraumatic sympathetic dystrophy, is a chronic neuropathic pain disorder characterized by autonomic findings and typically develops in an extremity after acute tissue trauma. In addition to classic neuropathic pain characteristics (intense burning pain, hyperalgesia, and allodynia), CRPS is associated with local edema and changes suggestive of autonomic involvement (altered sweating, skin color, and skin temperature in the affected region). Trophic changes to the skin, hair, and nails and altered motor function (loss of strength, decreased active range of motion, and tremors) may also occur. CRPS is subdivided to CRPS-I (reflex sympathetic dystrophy) and CRPS-II (causalgia), reflecting the absence or presence of documented nerve injury, respectively [1]. Although CRPS was first described in isolation, it can be linked to several diseases, such as trauma [1], psychiatric conditions [2], and cancer [3]. However, no case associated with Still’s disease has been previously described. Therefore, the objective of this study was to describe the first case of CRPS associated with Still’s disease. 2. Case Report A 50-year-old female began to complain in 2005 of polyarthritis of her knees, wrists, elbows, ankles, and hand metacarpophalangeal joints associated with fever, morning stiffness (for 4 hours), and evanescent rash. Laboratory results demonstrated leukocytosis, high levels of ferritin 401？ng/mL (reference value: 22–322？ng/mL), and erythrocyte sedimentation rate of 57？mm/1st hour. Antinuclear antibodies and rheumatoid factor were absent. Serologies for B and C hepatitis, HIV, HTLV 1 and 2, Epstein-Barr, rubella, toxoplasmosis, mononucleosis, rubella, and syphilis were negative. Echocardiography, liver and renal functions, myelogram, and bone marrow biopsy were also normal. A diagnosis of adult Still’s disease was performed,
References
[1]
A. Sharma, S. Agarwal, J. Broatch, and S. N. Raja, “A web-based cross-sectional epidemiological survey of complex regional pain syndrome,” Regional Anesthesia and Pain Medicine, vol. 34, no. 2, pp. 110–115, 2009.
[2]
A. Beerthuizen, D. L. Stronks, F. J.P.M. Huygen, J. Passchier, J. Klein, and A. V. Spijker, “The association between psychological factors and the development of complex regional pain syndrome type 1 (CRPS1)—a prospective multicenter study,” European Journal of Pain, vol. 15, no. 9, pp. 971–975, 2011.
[3]
N. Mekhail and L. Kapural, “Complex regional pain syndrome type I in cancer patients,” Current Review of Pain, vol. 4, no. 3, pp. 227–233, 2000.
[4]
K. Christensen, E. M. Jensen, and I. Noer, “The reflex dystrophy syndrome response to treatment with systemic corticosteroids,” Acta Chirurgica Scandinavica, vol. 148, no. 8, pp. 653–655, 1982.
[5]
C. Gobelet, M. Waldburger, and J. L. Meier, “The effect of adding calcitonin to physical treatment on reflex sympathetic dystrophy,” Pain, vol. 48, no. 2, pp. 171–175, 1992.
[6]
S. Adami, V. Fossaluzza, D. Gatti, E. Fracassi, and V. Braga, “Bisphosphonate therapy of reflex sympathetic dystrophy syndrome,” Annals of the Rheumatic Diseases, vol. 56, no. 3, pp. 201–204, 1997.
[7]
B. H. Lee, L. Scharff, N. F. Sethna et al., “Physical therapy and cognitive-behavioral treatment for complex regional pain syndromes,” Journal of Pediatrics, vol. 141, no. 1, pp. 135–140, 2002.
[8]
H. M. Oerlemans, R. A. B. Oostendorp, T. de Boo, and R. J. A. Goris, “Pain and reduced mobility in complex regional pain syndrome I: outcome of a prospective randomised controlled clinical trial of adjuvant physical therapy versus occupational therapy,” Pain, vol. 83, no. 1, pp. 77–83, 1999.
[9]
P. Sudeck, “Uè ber die akute (trophoneurotische) Knochenatrophie nach Entzuè ndungen und Traumen der Extremitaè ten,” Deutsche Medizinische Wochenschrift, vol. 28, pp. 336–342, 1902.
[10]
J. S. Calder, I. Holten, and R. M. R. McAllister, “Evidence for immune system involvement in reflex sympathetic dystrophy,” Journal of Hand Surgery, vol. 23, no. 2, pp. 147–150, 1998.
[11]
W. J. G. Oyen, I. E. Arntz, R. A. M. J. Claessens, J. W. M. van der Meer, F. H. M. Corstens, and R. J. A. Goris, “Reflex sympathetic dystrophy of the hand: an excessive inflammatory response?” Pain, vol. 55, no. 2, pp. 151–157, 1993.
[12]
J. C. Renier, J. Arlet, and C. Bregeon, “The joint in algodystrophy: joint fluid, synovium, cartilage,” Revue du Rhumatisme et des Maladies Osteo-Articulaires, vol. 50, no. 4, pp. 255–260, 1983.
[13]
M. Graif, M. E. Schweitzer, B. Marks, T. Matteucci, and S. Mandel, “Synovial effusion in reflex sympathetic dystrophy: an additional sign for diagnosis and staging,” Skeletal Radiology, vol. 27, no. 5, pp. 262–265, 1998.
[14]
M. Weber, F. Birklein, B. Neund？rfer, and M. Schmelz, “Facilitated neurogenic inflammation in complex regional pain syndrome,” Pain, vol. 91, no. 3, pp. 251–257, 2001.
