[Severe rhabdomyolysis revealing a myopathy linked to autoimmune hypothyroidism]. / Rhabdomyolyse sévère révélant une myopathie hypothyroïdienne d'origine auto-immune A propos de deux cas.

While muscular manifestations are common of hypothyroidism, hypothyroid myopathy is most often limited to myalgia, muscular stiffness and cramps with, in some patients, elevated levels of muscle enzymes. We report two cases of rhabdomyolysis related to hypothyroid myopathy. One of the patients developed acute renal failure. Thyroid hormone replacement therapy improved thyroid and renal function with involution of rhabdomyolysis. Hypothyroidism appears to be an authentic cause of rhabdomyolysis and should be carefully ruled out in all patients with elevated serum levels of muscle enzymes.

[Pseudo-tumoral and ischemic encephalic Erdheim-Chester disease]. / Maladie d'Erdheim-Chester cérébrale avec double lésion pseudotumorale et ischémique.

INTRODUCTION: Erdheim-Chester disease (ECD) is a rare non-langerhans cell histiocytosis of unknown etiology. It is a multi-systematic xanthogranulomatous infiltration with almost constant bone involvement; the neurological manifestations are not specific and occur in 15-20% of cases. METHODS: We report the case of a 59-year-old woman hospitalized for a frontal syndrome and right hemiparesis. RESULTS: Imaging revealed a left caudate nucleus process with recent infarct. Cardiovascular involvement and bilateral osteosclerosis of long bones strongly suggested ECD, confirmed after biopsies of the pericardium and bone. CONCLUSION: Pseudo-tumor encephalic ECD is very rare; the caudate nuclei is an unusual localization; ischemic stroke has been exceptionally described. Prognosis depends largely on the involvement of the central nervous and cardiovascular systems.

[Non-Hodgkin's lymphoma and hepatitis C virus infection]. / Lymphomes non hodgkiniens et infection par le virus de l'hépatite C.

INTRODUCTION: The authors report four cases of non Hodgkin's lymphomas (NHL) among patients infected by the hepatitis C virus (HCV). They discuss the epidemiological, physiopathological and clinical features of this association. CURRENT KNOWLEDGE AND KEY POINTS: The role of the HCV in the development of B-cell NHL is probable but the reported frequency of the association with HCV infection is variable especially in different countries. Most of the reported cases are low-grade NHL with frequent extranodal involvement. FUTURE PROSPECTS AND PROJECTS: New studies will improve the understanding of the physiopathological mechanisms that might explain the occurrence of NHL in the course of HCV infection. The recent evidence that the antiviral treatment is effective in splenic lymphoma with villous lymphocytes leads to reconsider the relationship between HCV and NHL and to think about new therapeutic possibilities.

[Non-Hodgkin's malignant lymhoma of mental soft tissue: a case report]. / Lymphome malin non hodgkinien (LMNH) des parties molles du menton. A propos d'un cas.

Non-Hodgkin's malignant lymphoma is rarely located in the soft tissue of the chin making diagnosis difficult. Clinical signs are non-specific: progressive weight loss, asthenia, fever, an inflammatory tumefaction unresponsive to medical treatment. Imaging shows a homogeneous tumefaction of the soft tissues. Pathology and immunohistochemistry establish the diagnosis.

[Sneddon's syndrome: 4 cases and a review of the literature]. / Le syndrome de Sneddon: 4 cas et revue de la littérature.

Sneddon syndrome is an association of livedo racemosa and cerebrovascular ischemic events generally occurring in young adults. This is an uncommon chronic progressive arterio-occlusive disorder of unknown cause involving small and medium sized vessels. We report four cases. One case was disclosed by cerebral hemorrhage. One pathogenic hypothesis suggests the involvement of an idiopathic progressive inflammatory arteriopathy or secondary thrombotic disorder comparable with antiphospholipid syndrome.

Idiopathic retroperitoneal fibrosis and ankylosing spondylitis. A new case report.

The case of a 52-year-old man with retroperitoneal fibrosis and ankylosing spondylitis is described. Inflammatory low back pain and acute renal insufficiency prompted a computed tomography scan of the abdomen with contrast agent injection. A fibrous sheath surrounding the aorta and attracting the ureters toward the midline was seen, strongly suggesting retroperitoneal fibrosis. The diagnosis of ankylosing spondylitis was based on the presence of inflammatory low back pain responsive to nonsteroidal anti-inflammatory drugs, syndesmophytes at the lumbar and cervical spine, bilateral sacroiliitis, and presence of the HLA-B27 antigen. Prednisone therapy in a daily dosage of 1 mg/kg induced a marked improvement. Only nine cases of concomitant retroperitoneal fibrosis and ankylosing spondylitis have been reported. These two conditions share similarities in some of the etiologic factors and anatomic localizations, suggesting that both may stem from a predisposition to fibrotic diseases.

[Heart and sports: modifications of electrocardiogram, late potentials and echocardiography. Study of 75 sportsmen and 46 witnesses]. / Coeur du sportif: modifications électriques et échocardiographiques au repos. Etude de 75 sportifs et de 45 témoins.

The authors report the results of prospective study, which compared 75 sports subjects and 45 witnesses. All subjects were male, the differentiating parameter being the type of sport practiced. This work analysed surface Electrocardiogramme, thransthoracic echocardiography and high amplification ECG. The statistical study used the student test t compare means and the Chi2 test for the percentages, the signification limit was fixed to 5%. Clinically, our two series didn't show a significant statistical difference, concerning: age, weight, height or arterial pressure. On the electrocardiographical level, the sport's men have a lower cardiac frequency (p = 0.005), a larger PR space (p = 0.05), an important Sokolow parameter (p < 0.005), and repolarisation disorders represented, essentially by negative T waves (p = 0.02) and an upper movement of ST segment in V2-V3 (p < 0.005). Echocardiography showed a dilatation of the right cavities: right auricular (p = 0.0125) and right ventricular (p = 0.025). Move over, it has been showed that the sport's men left ventricular walls were tabor (septal wall, p = 0.0125), (posterior wall, p = 0.025), despite a difference in the values of the left ventricular telediastolic diameter (4 mm average in the two series). The signification limit was not reached and it was also showed that the left auricular was also dilated (p = 0.025). The study of the delayed Potentials, tried to bring an explication to certain sudden deaths of sport's men which are to date unexplained and which could have a rhythmical origin? It is also to be noted that sport's men present more delayed ventricular Potentials. However, the statistical signification was not reached (p = 0.07).

[Digestive malacoplakia]. / Malacoplakie digestive.

INTRODUCTION: Malacoplakia, a chronic granulomatous disease, rarely involves the digestive tube and, when it does, takes on a pseudotumoral aspect. OBSERVATION: A 37 year-old man was hospitalized for chronic diarrhea that had progressed over 15 years. He exhibited an edematous-ascitic syndrome and bilateral pleurisy together with, biologically, a malabsorption syndrome. The endoscopic examinations (fibroscopy and colonoscopy) revealed polypoid tumor-like formations. An image of tumoral stenosis of the sigmoid-colic junction was revealed on barite lavage. Histological examination of the surgical sample (wide left colectomy of one third of the transverse) was suggestive of malacoplakia (histiocytes with characteristic Michaelis-Gutmann bodies). DISCUSSION: Malacoplakia is an inflammatory disease predominantly affecting the urogenital tract. Other organs can be involved. Its clinical symptomatology is highly disparate. The interest of this disease is in its pathogenesis, on which, in fact, its treatment is based.

[Biermer's disease and venous thrombosis. Report of two cases]. / Maladie de Biermer et thrombose veineuse. A propos de deux observations.

INTRODUCTION: The main issue in venous thrombotic events is their etiology. Several clinical and/or biological data can be helpful in that research. In the two cases we report here, a macrocytic anemia led to the diagnosis of probably acquired hyperhomocysteinemia. FIRST CASE: a 24-year-old man was admitted for severe anemia and a superior vena cava syndrome. Biological data showed megaloblastic anemia and mild features of blood destruction that were explained by a pernicious anemia. Second case: a 35-year-old man had two deep venous thrombotic events in one year (involving the right leg, then the left leg); biological findings showed a macrocytic mild anemia that was diagnosed as a pernicious anemia. In both of the patients, deep venous thrombosis was mainly explained by a hyperhomocysteinemia that was a consequence of vitamin B12 deficiency. The two patients improved under anticoagulant treatment combined with subcutaneous vitamin B12. CONCLUSION: Pernicious anemia can cause acquired hyperhomocysteinemia, which is considered a risk factor for deep venous thrombosis. Thus, the connectivity of these conditions should remain in the practitioner's mind, especially when thrombosis occurs along with a macrocytic anemia.

[What is new in antithrombotic treatment?]. / Quoi de neuf dans le traitement antithrombotique?

Real progresses have been made during the past years in the comprehension of hemostasis mechanisms, along with rising of new antithrombotic drugs. The later include: 1) direct inhibitors of thrombin such as hirudin, bivalirudin, argatroban, melagatran and ximelagatran; 2) inhibitors of factor Xa such as the synthetic pentasaccharid and DX-9065a; 3) inhibitors of factor IXa; 4) inhibitors of tissue factor-factor VIIa complex such as tissue factor pathway inhibitor (TFPI) or NAPc2 (nematode anticoagulant peptide); 5) drugs enhancing endogenous anticoagulant activity, such as protein C or activated protein C; 6) drugs modulating endogenous fibrinolytic activity. These new drugs are promising a real decrease in mortality and morbidity due to venous thrombo-embolic disease, which is considered as a public health issue. Both physicians and biologists are concerned by these new antithrombotic agents, the former to think about new treatment strategies, the later to monitore, if necessary, the effects of such new drugs. Our review does not include antiplatelet agents which are indicated only in arterial thrombosis.

A controlled study of sacroiliitis in Behçet's disease.

The aim of the study was to evaluate the prevalence of sacroiliitis in a group of patients with Behçet's disease (BD). Pelvic X-rays of 27 patients with BD responding to the International Study Group of BD and 30 controls (15 AS and 15 sciatica) were read blind and sacroiliac involvement was graded according to the New York criteria. In a second step, patients or controls with equivocal sacroiliitis had a sacroiliac CT scan. Two patients with BD (7.4%) and all patients with AS had evident bilateral sacroiliitis (at least grade 2). One patient with BD and two patients with sciatica had equivocal sacroiliitis (grade 1). CT confirmed sacroiliitis in the two patients with BD and eliminated inflammatory sacroiliitis in the three other patients with equivocal sacroiliitis showing mild degenerative lesions. A review of the literature showed that sacroiliitis and AS are rarely associated with BD. There remains insufficient evidence to suggest that sacroiliitis is an intrinsic feature of BD and that BD belongs to the group of SpA.

[Endomyocardial fibrosis in Behcet's disease: a case report of a pseudo-tumoral form]. / Fibrose endomyocardique au cours de la maladie de Behçet: un cas à forme pseudo-tumorale.

Endomyocardial fibrosis is very rare in Behcet's disease. The authors report the case of a 28 year old patient with Behcet's disease complicated by a pseudo-tumoral right ventricular formation on echocardiography. This misleading appearance suggested the diagnosis of cardiac thrombus or tumour and led to a surgical approach which revealed a fibrous moderator band suggesting endomyocardial fibrosis, confirmed by antomopathological analysis. Besides the originality of this case and the unusual pseudo-tumoral presentation, the authors underline the difficulties of establishing the diagnosis, despite the advances of medical imaging. The pseudo-tumoral intra-cardiac lesion in a suggestive clinical context (Behcet's disease) should raise suspicion of the diagnosis of endomyocardial fibrosis.

[McDuffie hypocomplementemic urticarial vasculitis. Two cases and review of the literature]. / Vascularite urticarienne hypocomplémentémique de McDuffie. Deux observations et revue de la littérature.

INTRODUCTION: Hypocomplementemic urticarial vasculitis (HUV) described by McDuffie is a rare entity recently individualized among vasculitis. We report two new cases. EXEGESIS: Case 1: a 41-year-old woman presented in 1994 with inflammatory polyarthralgia, diffuse urticaria, fever, and weight loss. Biology showed proteinuria, positive rheumatoid factor with hypocomplementemia and negative immunological tests. Skin and renal biopsies showed leukocytoclastic vasculitis and extramembranous glomerulopathy, respectively. Outcome within steroid therapy was marked by alternating clinical improvement and relapses. Case 2: a 39-year-old woman presented in 1994 with inflammatory polyarthritis, diffuse urticaria, Raynaud phenomenon, cough and dyspnea. Chest x-rays and CT scan showed interstitial fibrosis and echocardiography revealed pericarditis. Biology showed positive rheumatoid factor with hypocomplementemia and negative antinuclear antibodies. Skin biopsy showed leukocytoclastic vasculitis. Corticosteroids and cyclophosphamide improved the patient's condition. McDuffie HUV is a disease with varied systemic manifestations. Its existence is still contested by some authors. Treatment is still empirical and depends on the clinical features. It is based primarily on corticosteroids. CONCLUSION: McDuffie HUV is a defensible entity among urticarial vasculitis because of its particular clinical and biological features.

[Systemic manifestations of Sweet's syndrome: a case report]. / Manifestations systémiques du syndrome de Sweet. A propos d'un cas.

Sweet's syndrome belongs to the group of neutrophilic dermatoses. We report the case of a 36-year-old man admitted for stiff neck and fever. He had a history of recurrent oral aphtous ulcers, orchitis, phlebitis, two episodes of febrile acute polyarthritis with interstitial pneumonia. He presented a stiff neck and a temperature of 40 degrees C for two days associated with an erythematonodular eruption of the right periocular region. Laboratory exams showed an inflammatory syndrome with hyperleukocytosis. Skin biopsy showed dermic neutrophilic infiltrates, confirming the diagnosis of Sweet's syndrome. The patient improved dramatically with corticosteroids: the temperature fell and neck stiffness and skin lesions disappeared. In light of this case with a rich cohort of extracutaneous manifestations, we reviewed the literature on the characteristics of Sweet's syndrome. This syndrome is commonly associated with inflammatory and neoplastic diseases.