[Seronegative antiphospholipid syndrome, catastrophic syndrome, new anticoagulants: learning from a difficult case report]. / Syndrome des antiphospholides "séronégatif ", syndrome catastrophique, nouveaux anticoagulants: enseignements d'une observation de prise en charge difficile.

INTRODUCTION: The diagnosis of the antiphospholipid syndrome (APS) is based on clinical and biological criteria including the persistent presence of antiphospholipid antibodies and thrombotic events or pregnancy morbidity. Heparins relayed by vitamin K antagonists (VKA) are the gold standard treatment for thrombosis. CASE REPORT: We report a 17-year-old man who presented with an initially seronegative antiphospholipid syndrome, in whom the diagnosis was late, only obtained after anticoagulation withdrawing, when a catastrophic antiphospholipid syndrome (CAPS) with cutaneous lesions and disseminated intravascular coagulation syndrome occurred. For personal convenience, this patient was initially treated with fondaparinux followed by a new oral anticoagulant (rivaroxaban) before to return to the conventional VKA treatment. CONCLUSION: The "seronegative" APS is a controversial concept reflecting the heterogeneity of antigenic targets for aPL. This diagnosis may be considered after a rigorous work-up, with the help of haemostasis laboratories testing new emerging aPL assays. In APS, the new anticoagulants represent an attractive option needing nevertheless prospective studies to evaluate their safety and efficacy. Lupus anticoagulant detection in patients treated by new oral anticoagulants is not easy by usually recommended coagulation tests.

[Pyoderma gangrenosum, subcorneal pustular dermatosis and aseptic spleen abscess: "a neutrophilic disease"]. / Association pyoderma gangrenosum, pustulose sous-cornée et abcès aseptiques spléniques: "une maladie neutrophilique".

Neutrophilic diseases may have various clinical presentations but share common histopathological manifestations with an aseptic infiltrate of polymorphonuclears neutrophils. The association with various diseases is frequent and must be systematically discussed. We report a patient who presented three different synchronous clinical manifestations of neutrophilic disease: pyoderma gangrenosum, subcorneal pustular dermatosis and aseptic spleen abscesses.

[Actinomyces odontolyticus isolation during prosthetic aortic graft infection with paraprosthetic duodenal fistula]. / Isolement d'Actinomyces odontolyticus au cours d'une infection de prothèse aortique avec fistule duodénoparaprothétique.

INTRODUCTION: Prosthetic vascular graft infection is a rare complication of vascular surgery. We report a case with graft enteric fistula and Actinomyces odontolyticus bacteremia. EXEGESIS: A 73 year-old man with a prosthetic aortic graft and who had a parodontal disease, has been hospitalised for fever of unknown origin. Blood cultures grew with Escherichia coli and Actinomyces odontolyticus. The imaging studies indicated graft infection. Laparotomy has confirmed the diagnosis and highlighted a polymicrobial infection and a paraprosthetic duodenal fistula. A review of the literature's data concerning prosthetic vascular graft infections is made. The role of Actinomyces odontolyticus in that case is discussed. CONCLUSION: Prosthetic aortic graft infection due to graft enteric fistula is usually a polymicrobial infection and is a late complication of aortic surgery. Imaging is essential for the diagnosis of prosthetic aortic graft infection. It is possible that Actinomyces odontolyticus has contributed to prosthesis infection in this case.

Subacute bacterial endocarditis presenting as polymyalgia rheumatica or giant cell arteritis.

OBJECTIVE: To report on several patients with subacute bacterial endocarditis who were initially presumed, incorrectly, to have polymyalgia rheumatica or giant cell arteritis. METHODS: We report 3 cases of subacute streptococcal endocarditis mimicking giant cell arteritis in 2 cases and polymyalgia rheumatica in one. We reviewed the literature through Medline search of French and English-language articles published between 1966 and 2005 and found 5 similar cases. RESULTS: Shoulder and/or pelvic girdle pain was associated with neck or back pain in all patients. Scalp tenderness, bilateral jaw pain, amaurosis fugax were present in 2 patients. One patient had no fever. Two patients were treated with corticosteroids with initial good clinical response in one. Appropriate antibiotic therapy resulted in the rapid disappearance of rheumatic complaints in 2 patients and achieved a definitive cure of endocarditis in all cases. CONCLUSION: Rheumatologic symptoms may hinder the correct diagnosis of infective endocarditis in patients who present with a clinical picture suggesting polymyalgia rheumatica or giant cell arteritis. In such cases, blood cultures should be systematically drawn.

[Prosthetic valve endocarditis due to Coxiella burnetii: six cases]. / Endocardites à Coxiella burnetii sur prothèse: six observations.

PURPOSE: Prosthetic valve endocarditis is a dangerous complication of valvular surgery (3-6%). Among involved pathogens, Coxiella burnetii is an occasional agent, though isolated with increasing frequency. We report our experience with this peculiar endocarditis and lay stress on specific diagnostic and therapeutic difficulties. METHODS: Between 1990 and 1995, six patients retrospectively met the diagnosis criteria for definite endocarditis due to Coxiella burnetii. RESULTS: Five Algerian men and one French woman presented with prosthetic valve endocarditis with negative blood cultures (on bioprosthesis: four cases, on mechanical valve: two cases). The main clinical and biological feature was febrile congestive heart failure with hepatomegaly, splenomegaly, hepatic and renal abnormalities, inflammatory syndrome, hypergammaglobulinemia, anemia and lymphopenia. Serological testing for Coxiella burnetii provided diagnosis in all cases. Echocardiography displayed vegetations in all cases. Valvular replacement was performed in four patients. With antibiotic therapy including doxycycline or/and hydroxychloroquine, quinolones or rifampicine, all patients experienced complete clinical, biological and echographic remission. CONCLUSION: Q fever prosthetic valve endocarditis presents as a systemic disorder occurring in patients with valvular heart disease. From now on, early diagnosis and efficient medical treatment may provide permanent prosthetic sterilization.

Pregnancy in patients with Wegener's granulomatosis: report of five cases in three women.

Five cases of pregnancy occurring in three women with previously diagnosed Wegener's granulomatosis are described. The disease was diffuse in one case and localised in the other. Initial treatment consisted of a combination of corticosteroids and intravenous cyclophosphamide in two women, and methotrexate in one. Four pregnancies ended in live births despite pre-eclampsia in two cases. One therapeutic abortion was induced because of encephalocele. Comparable reported cases were reviewed to examine the implications of immunosuppressive treatment on the fetus. A relapse occurred during pregnancy in 40% of the cases, but in 25% if only pregnancies beginning during inactive disease were taken into account. No other indicator for maternal and fetal outcome was obvious. Pregnancy should be planned after complete disappearance of disease activity. In the case of a relapse a combination of immunosuppressive drugs and corticosteroids should be chosen rather than corticosteroids alone because the outcome of pregnancy is poor in cases of undertreatment. Prematurity remains common.

[Polymyositis and essential thrombocytopenia. A case report]. / Polymyosite associée à une thrombocytémie essentielle.

The occurrence of polymyositis (PM) correlates with an increased risk of solid tumor. Among hematologic malignancies that are sporadically associated with PM or dermatopolymyositis (DM), lymphoma and chronic lymphoid leukemia are the most frequent. The association between PM and myeloproliferative disorders remains exceptional. We describe the simultaneous revelation of a PM and an essential thrombocytemia (ET). The sensitivity of the PM to corticosteroids was noteworthy and allowed a quick and definitive control of clinical and biological inflammation. Despite the efficiency of a cytostatic agent upon the thrombocytemia, the patient died after numerous thrombotic and hemorrhagic complications of ET. This is the first published case of such an association between PM and TE. The paraneoplastic significance of the PM is discussed.

Localized subcutaneous Nocardia farcinica abscess in a woman with overlap syndrome between systemic scleroderma and polymyositis.

Nocardia farcinica is an increasingly common cause of human infection in Europe. We observed an isolated nodular subcutaneous abscess due to N. farcinica in the forearm of a young patient with overlap syndrome between systemic sclerosis and polymyositis. Despite in vitro resistance to trimethoprim-sulfamethoxazole, this oral combination completely resolved the lesion within 3 weeks and was maintained for 6 months. The spectrum of cutaneous nocardiosis in the immunocompromised host is discussed.