Aggressive angiomyxoma (AAM) is a rare tumor that preferentially involves the pelvis and perineal regions and arises from the connective tissue. Its cause and pathogenesis are unknown at present. Treatment typically involves surgery, and despite apparently complete resection, local recurrences are common. We describe a case of a large angiomyxoma of the left pelvis in a 59-year-old woman who underwent two surgical excisions. The first had been done in May 1998. She developed a local recurrence in December 1998. A palliative resection with macroscopic residuals was performed in February 2001, followed by radiation therapy with a total dose of 60 Gy. The diagnosis was revised at the time of the second operation. Initially, the tumor was diagnosed as angiomyofibroblastoma. Follow-up 3 years after the radiation treatment revealed no recurrence. The time of the local control achieved as yet is already longer than the former time to progression between the first two surgical procedures. This is, to our knowledge, the second description of a therapeutic irradiation of a recurrent AAM. Radiation therapy is able to control a recurrent AAM for at least 3 years.

Accurate and sensitive measurement techniques are a key issue in the quantification of the microvascular and endothelial dysfunction in systemic sclerosis (SSc). Thermal hyperhemia comprises two separate mechanisms: an initial peak that is axon reflex mediated; and a sustained plateau phase that is nitric oxide dependent. The main objective of our study was to test whether thermal hyperhemia in patients with SSc differed from that in patients with primary Raynaud's phenomenon (RP) and healthy controls. In a first study, we enrolled 20 patients suffering from SSc, 20 patients with primary RP and 20 healthy volunteers. All subjects were in a fasting state. Post-occlusive hyperhemia, 0.4 mg sublingual nitroglycerin challenge and thermal hyperhemia were performed using laser Doppler flowmetry on the distal pad of the third left finger. In a second study, thermal hyperhemia was performed in 10 patients with rheumatoid arthritis and 10 patients with primary RP. The thermal hyperhemia was dramatically altered in terms of amplitude and kinetics in patients with SSc. Whereas 19 healthy volunteers and 18 patients with primary RP exhibited the classic response, including an initial peak within the first 10 minutes followed by a nadir and a second peak, this occurred only in four of the SSc patients (p < 0.0001). The 10 minutes thermal peak was 43.4 (23.2 to 63), 42.6 (31 to 80.7) and 27 (14.7 to 51.4) mV/mm Hg in the healthy volunteers, primary RP and SSc groups, respectively (p = 0.01), while the 44°C thermal peak was 43.1 (21.3 to 62.1), 42.6 (31.6 to 74.3) and 25.4 (15 to 52.4) mV/mm Hg, respectively (p = 0.01). Thermal hyperhemia was more sensitive and specific than post-occlusive hyperhemia for differentiating SSc from primary RP. In patients with rheumatoid arthritis, thermal hyperhemia was also altered in terms of amplitude. Thermal hyperhemia is dramatically altered in patients with secondary RP in comparison with subjects with primary RP. Further studies are required to determine the mechanisms of this altered response, and whether it may provide additional information in a clinical setting.

1. International Study Group for Behçet’s disease. Criteria for diagnosis of Behçet’s disease. Lancet 1990;335:1078–80. 2. O’Day DM, Horn JD. The eye and rheumatic disease. In: Ruddy S, Harris ED, Sledge CB, eds. Kelley’s Textbook of rheumatology. Philadelphia: Saunders, 2001:393–400. 3. Kaklamani VG, Kaklamanis PG. Treatment of Behçet’s disease: an update. Semin Arthritis Rheum 2001;30:299–312. 4. Robertson LP, Hickling P. Treatment of recalcitrant orogenital ulceration of Behçet’s disease with infliximab. Rheumatology 2001;40:473–4. 5. Triolo G, Valada M, Accardo-Palumbo A et al. Anti-tumour necrosis factor monoclonal antibody treatment for ocular Behçet’s disease. Ann Rheum Dis 2002;61:560–1. 6. Sfikakis PP, Kaklamanis PH, Elezoglou A et al. Infliximab for recurrent, sight-threatening ocular inflammation in AdamantiadesBehçet disease. Ann Intern Med 2004;140:404–6. 7. Ohno S, Nakamura S, Hori S et al. Efficacy, safety, and pharmacokinetics of multiple administration of infliximab in Behçet’s disease with refractory uveoretinitis. J Rheumatol 2004;31:1362–8. 8. Pavesio CE, Meier FM. Systemic disorders associated with episcleritis and scleritis. Curr Opin Ophthalmol 2001;12:471–8. 9. Nguyen QD. Scleral patch graft in the management of necrotizing scleritis. Int Ophthalmol Clin 1999;39:109–31. 10. Murphy CC, Ayliffe WE, Booth A, Makanjuola D, Andrews PA, Jayne D. Tumor necrosis factor blockade with infliximab for refractory uveitis and scleritis. Ophthalmology 2004;111:352–6.

Nous rapportons le cas rare d’une épidurite infectieuse à Staphylococcus aureus cervico-dorso-lombaire. Le diagnostic a été difficile à établir sur la première IRM. L’évolution clinique a été favorable sous traitement médical avec régression complète des symptômes après un recul de un an.

We report a case of Staphylococcus aureus epidural infection extending from the cervical to the lumbar spine. Findings from the first magnetic resonance imaging study were misleading. The symptoms resolved fully under medical treatment, with no recurrence after 1 year.

Nous rapportons six cas d'epidurites infectieuses observees entre 1986 et 1999, dont un cas de pan-epidurite Nous comparons les caracteristiques anamnestiques, cliniques, biologiques, therapeutiques et radiologiques. Le mode de diffusion est hematogene dans tous les cas, sans contamination directe, ni de facteur predisposant. Tous les patients ont presente un tableau de lombalgie febrile avec des signes neurologiques soit moteurs soit sphincteriens, rarement sensitifs La latence diagnostique est variable, de 48 heures a 3 semaines. Les donnees biologiques sont aspecifiques et orientent simplement vers une origine potentiellement infectieuse. Les germes (Staphylocoque aureus (50%), Streptocoque (33%) et Haemophilus aphrophilus (1 cas) ont ete identifies grâce aux hemocultures (80%) et grâce a la ponction discale dans 1 cas. Les radiographies et la scintigraphie osseuse ont peu d'interet diagnostique. Nous nous attachons dans cette etude a souligner l'interet de l'IRM a titre diagnostique, mais aussi a titre pronostique. Elle montre une coulee ou une masse siegeant dans l'espace epidural, iso-intense (a la moelle) ou hypo-intense en T1, se renforcant apres injection de Gadolinium et hyperintense en mode T2. Pour trois patients, apres un mois de recul, les anomalies de signaux a l'IRM persistent alors que l'evolution clinique et biologique est favorable. De plus, nous proposons une conduite a tenir pour la surveillance des epidurites infectieuses: IRM T1, T1 gadolinium et T2, a une semaine, puis entre 4 a 6 semaines Une troisieme IRM est indiquee a 10-12 semaines si persistance de signes cliniques, biologiques et d'image d'abces a l'IRM de la sixieme semaine.