[Scleroderma-like disorders]. / Les syndromes sclérodermiformes.

The finding of hardening and thickening of the skin is common and can be encountered in immune mediated, metabolic, neoplastic, toxic, genetic diseases, or associated with protein deposits. The lack of Raynaud's phenomenon, capillaroscopic abnormalities, or scleroderma-specific autoantibodies should question the diagnosis of scleroderma and trigger the search for a scleroderma-like disorder, for which treatment and prognosis differ. This article gives a review of these disorders and their main characteristics.

[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.

[Lymphomatoid granulomatosis]. / Granulomatose lymphomatoïde.

Lymphomatoid granulomatosis, described in 1972 by Liebow, is a rare, Epstein-Barr virus (EBV)-associated lymphoproliferative disorder, involving the lung, and often the skin or the central nervous system. It could have a systemic course making its diagnosis difficult. Controversy still remains about precise taxonomy and lymphomatoid granulomatosis is classified as a lymphoma, whose severity extends from indolent forms to aggressive large B cell lymphomas. Histology is essential and shows characteristically an inflammatory angiocentric infiltrate, composed with polymorphous mononucleated cells containing a varying number of large atypical CD20-positive B-lymphocytes within a background of numerous small reactive CD3-positive T-lymphocytes, often associated with necrosis. In situ hybridization often shows EBV RNA within atypical B-cells. Atypical large B-lymphocytes proportion and to a lesser degree EBV-positive B-lymphocytes proportion allow to classify the disease (grade I to III) and have a prognostic value. An aggressive form of B lymphoma occurs in 7 to 47% of cases during lymphomatoid granulomatosis course. Moreover, grade III diseases share numerous characteristics of lymphoma and often require chemotherapy. Several conditions mimic lymphomatoid granulomatosis, and include various hematologic malignancies (large B-cells lymphomas, T/NK lymphomas, post-immunodepression lymphoproliferative disorders) or granulomatosis with polyangiitis. The objective of this article is to review the clinical, radiological, histological and therapeutic characteristics of this rare disorder.

[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.

Mortality among human immunodeficiency virus-infected patients with cirrhosis or hepatocellular carcinoma due to hepatitis C virus in French Departments of Internal Medicine/Infectious Diseases, in 1995 and 1997.

Several studies have suggested that the progression of hepatitis C virus (HCV) infection is more severe in patients infected by the human immunodeficiency virus (HIV). Two national retrospective multicenter cohort surveys were performed in France that included 17,487 HIV-infected patients during 1995 and 26,497 during 1997. The following data was evaluated: total number of deaths; number of deaths linked to AIDS, cirrhosis, or hepatocellular carcinoma (HCC); and number of deaths related to other (non-HCV--linked) causes. In 1995, the causes of death were as follows: AIDS, 1307 (7.47%); cirrhosis or HCC, 21 (0.12%); and other (non-HCV--linked) causes, 99 (0.56%). In 1997, the causes of deaths were as follows: AIDS, 459 (1.73%); cirrhosis or HCC 36 (0.13%); and other (non-HCV--linked) causes, 48 (0.18%). Comparative results between the 1995 and 1997 surveys showed a dramatic decrease in AIDS-related mortality rates (7.47% vs. 1.73%; P<.001) but not in HCV-related mortality rates (0.06% vs. 0.07%; P=.79). In France, despite the high prevalence of HCV infection in HIV-positive patients, the mortality rate in 1995 and 1997 caused by HCV-related cirrhosis or HCC was low.

[Infections associated with pets]. / Infections transmises par les animaux de compagnie.

INTRODUCTION: Domestic pets can transmit numerous infections, including bacterial, parasitic, fungal, and viral diseases. This paper reports the epidemiologic, clinical, therapeutic and prophylactic data of these zoonoses. CURRENT KNOWLEDGE AND KEY POINTS: The routes of transmission are various. Bites and scratches are the most common health hazards and result in localized infections. Pasteurellosis, various aerobic and anaerobic infections, and cat-scratch disease are predominant. Bites are treated by cleaning the wound, rabies and tetanus prophylaxis, and the appropriate use of antibiotics. Other infections are transmitted through cutaneous, mucous, digestive or respiratory routes, by direct contact with the pets, excreta, or by arthropods. The most common are gastrointestinal (campylobacter, salmonella, yersinia, parasites, etc), dermatologic (dermatophytoses, scabies, cutaneous larva migraines, etc.), respiratory (psittacosis, etc.), and multisystemic (toxoplasmosis, toxocariasis, leishmaniosis). Certain people are at high risk for diverse diseases: small children (toxocariasis, helminthiasis), pregnant women (toxoplasmosis), and immunodeficient patients (cryptosporidiosis, salmonellosis, systemic pasteurellosis). These infectious diseases can be partly prevented by avoiding contact with diseased animals, and by washing the hands following exposure to pets or pet-derived excreta. Specific vaccines for humans and pets, as well as worming pets regularly, form an important part of the prevention. Veterinarians must discourage the keeping of wild or exotic animals as pets. FUTURE PROSPECTS AND PROJECTS: National health survey institutions and new communication systems can improve our knowledge about the real epidemiology of pet-transmitted zoonoses.