[Axillary lymphadenitis due to Corynebacterium pseudotuberculosis in a 63-year-old patient]. / Lymphadénite axillaire à Corynebacterium pseudotuberculosis chez une patiente de 63 ans.

A 63-year-old woman living in a rural area presented in 2004 with a granulomatous necrotizing axillary lymphadenitis caused by Corynebacterium pseudotuberculosis, a Gram positive bacillus belonging to the group of Corynebacterium diphtheriae, which is found primarily in animal infections. In the human being, around 30 cases of infection due to C. pseudotuberculosis have been described, mainly among people working in contact with infected animals. For this reason, this infection, which induces exclusively lymphadenitis and abscesses with granulomatous necrotizing lesions, should be regarded as an occupational disease. In this patient, due to ignorance of the disease, this bacterium was considered to be merely saprophytic. The first treatment introduced was probabilistic antibiotherapy followed by a second course of antibiotics after aspiration. The trend confirmed that this was not the correct treatment and that surgery was the only way to achieve a cure.

[Serogroup C meningococcemia presenting as pericarditis in a 55-year-old woman without medical history]. / Péricardite purulente compliquant une méningococcémie du groupe C chez une femme de 55 ans sans antécédent notable.

In case of meningococcemia, isolated pericarditis is a rare event with a high mortality rate. We report the clinical and paraclinical signs that lead us to the diagnosis in a 55-year-old woman with favourable evolution. Previously described predisposing factors were no found.

[Cerebral-vein thrombosis: retrospective study of twenty seven cases]. / Thrombophlébite cérébrale: étude rétrospective de vingt-sept cas.

PURPOSE: Among the locations of venous thrombosis, even if rare, cerebral-vein thrombosis is a severe event with a high mortality rate. No aetiology is found in 20 to 30% of the cases. In recent years, inherited coagulation disorders have been described, as risk factors for venous thrombosis. We report the results of a retrospective study of 27 patients who suffered cerebral-vein thrombosis, in which coagulation abnormalities have been searched for. METHOD: The patients were referred to the haemostasis laboratory of the Henri Mondor hospital between august 1982 and June 1988, after a cerebral-vein thrombosis. The predisposing factors, personal and family history of thromboembolism, clinical presentation, thrombosis location, evolution under treatment and long-term outcome, have been noted. Deficiencies in antithrombine, protein C, protein S, the Factor V Leiden and the G20210A prothrombin-gene mutation, the presence of lupus anticoagulant, of anticardiolipin antibodies as well as a hyperhomocysteinaemia have been searched, either at the initial presentation, or a posteriori. RESULTS: Fourty-one percent of patients had a coagulation abnormality. The prevalence of the different abnormalities was: inherited deficiency in AT 7.4%, in PC 8%, in PS 12.5%, factor V Leiden mutation 12%, G20210A prothrombin-gene mutation 12%. Two patients had combined defects: AT and PC deficiency in one, F V Leiden and F II G20210A mutations in one. e of the patient had lupus anticoagulant. Three patients had a significant rate of anticardiolipin antibodies. Five patients out of eight displayed a moderate hyperhomocysteinaemia. Nothing (past history, age, predisposing factors) distinguished those patients bearing a coagulation disorder from the others. The venous thromboembolic relapse rate of 15 % (4/27 patients). Three of them had an inherited thrombophilic abnormality. CONCLUSION: We recommend an investigation of the haemostasis after every cerebral venous thrombosis.

Embolized ischemic lesions of toes in an afibrinogenemic patient: possible relevance to in vivo circulating thrombin.

Fibrinogen plays a complex role in hemostasis, thrombosis, and vascular disease. Hyperfibrinogenemia is an independent vascular risk factor and dysfibrinogenemia can provoke thrombosis. Afibrinogenemia is usually responsible for hemorrhagic diathesis, and unexpected ischemic lesions are intriguing. We report the case of an afibrinogenemic patient, who at the age of 30 developed ischemic lesions of the feet related to severe stenosis of the iliac and hypogastric arteries. The biopsy of the iliac artery lesion showed an intense myointimal hyperplasia. We performed standard hemostatic analysis and analyzed the activation markers of platelets and coagulation factors and the kinetics of thrombin generation in the patient and in normal control plasmas treated or not with reptilase. Occlusive arterial lesions were attributed to a disruptive hematoma penetrating the vascular lumen. Thrombin concentration after calcium addition increase markedly in the afibrinogenemic patient and in defibrinated normal plasma, as compared to untreated normal plasma. Thrombin-antithrombin complexes (T-AT) were markedly enhanced while F1+2 prothrombin fragments stayed in the normal range. These results suggested activation of coagulation and in vivo circulating thrombin. Thrombin activates the platelets that secrete growth factors for smooth muscle cells and generate the intimal hyperplasia. Recurrent hemorrhage within the vessel wall might induce injury and local thrombin generation. Thrombin not trapped by the clot is available for platelet activation and smooth muscle cell migration and proliferation. The absence of a protective fibrin cap on the intima might account for intima vulnerability and embolization. Afibrinogenemia appears in this paradoxical situation as a vascular risk factor.

[Behcet's disease manifested as a intracranial aneurysm]. / Maladie de Behçet révélée par un anévrisme intracrânien.

INTRODUCTION: Mostly venous (95% of all vascular complications), and less frequently arterial (2 to 7% of all cases), vascular complications are commonplace in Behçet's disease (23 to 64% of the patients, depending on the series). Arterial complications are stenosis, occlusions and especially severe due to their unpredictable rupture risk, aneurysms. Intracranial aneurysms associated with Behçet's disease are exceptional. Until now, only ten cases have been published. EXEGESIS: We report the case of a 36-year-old patient of Armenian origin in whom the diagnosis of Behçet's disease was made after a subarachnoid hemorrhage caused by the rupture of a left superior cerebellar artery aneurysm. The endovascular treatment of the aneurysm was associated with an immunosuppressive treatment consisting of cyclophosphamide, corticoids and colchicine. Within a 6-month period of follow up the evolution has been favorable. This is the first published case report of Behçet's disease associated with an aneurysm of the posterior circulation treated endovascularly. A review of the literature is also included. CONCLUSION: Intracranial aneurysms are an exceptional but nevertheless severe localization of vascular complications in Behçet's disease. As in all other arterial lesions, recurrences are frequent. The treatment involves surgical or endovascular treatment that should be associated with corticoids and immunosuppressive therapy. Colchicine is useful for the prevention of relapses.