Une télangiectasie hémorragique héréditaire révélée tardivement par une thrombose veineuse cérébrale : à propos d'un cas

IntroductionHereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease characterized by the triad of nose bleeding, telangiectasia and familial heredity.Case reportWe report the case of a patient who had idiopathic venous cerebral thrombosis complicated by a cerebral infarction treated with warfarin. In the context of a psoas hematoma by warfarine overdose and immobilization, the patient had deep vein thrombosis of the left lower limb with pulmonary embolism revealing a pulmonary arteriovenous malformation. After a reexamination, the patient clinical phenotype of HHT was confirmed genetically. The patient was treated with rivaroxaban allowing clinical improvement and partial recanalization of all thrombosis after six months. Thrombotic overisk has already been studied in HHT patients but the use of anticoagulants is at higher risk in these patients. However this patient experienced no adverse event with rivaroxaban.ConclusionThis is the first case described of cerebral venous thrombosis treated with rivaroxaban revealing an HHT.     

Méningite lymphocytaire chez une patiente lupique révélant un lymphome cérébral primitif : une association rare

Introduction

We report an unusual observation of central nervous system (CNS) lymphoma in a 60-year-old woman with systemic lupus erythematosus and fatal outcome.

Myalgies fébriles révélant une endocardite infectieuse à Propionibacterium acnes

IntroductionPropionibacterium acnes endocarditis is rare and difficult to diagnose. We report a case of Propioniacterium acnes endocarditis revealed by a lower limb fasciitis.Case reportA 54-year-old patient presented with recurrent febrile myalgia of the lower limbs, that appeared three years after an aortic surgery (aortic valve sparing reimplentation and ascending aortic prosthesis implantation). Computer tomography showed fasciitis of both legs. Positron emission tomography showed ¹⁸Fluorodeoxyglucose intake of the aortic prosthesis and in muscles of the lower limbs. Ten days after blood sample drawing, cultures showed the presence of Propionibacterium acnes. The aortic prosthesis was surgically removed, whose culture confirmed infection by Propionibacterium acnes. The diagnosis of infective endocarditis revealed by lower limb emboli was made. Evolution was favorable.ConclusionIn patients with vascular prostheses, Propionibacterium acnes infection must be evoked face to an atypical inflammatory process. Very prolonged blood culture incubation is needed to identify the pathogen.     

Une ischémie critique du membre inférieur chez une jeune femme révélant la maladie du Lupus

The diversity of juvenile arterial disease aetiologies requires a systematic investigation when limb critical ischemia is diagnosed before the age of 50. We would like to share a rare case of a young woman who was diagnosed with Lupus disease revealed by distal ischemia of the foot.     

Une ascite révélant un lymphome non hodgkinien de type B et une hépatite chronique virale B

Hepatic localization of non-Hodgkin lymphoma is generally secondary, B or T-cell lymphoma, according to the classification by the World Health Organization. The liver involvement in lymphoma corresponds to stage IV of the Ann Arbor staging classification. The symptomatology is varied. Cytolysis and/or cholestasis can occur. Liver biopsy associated with an immunohistochemistry study confirms the diagnosis. Treatment consists of a polychemotherapy. We report the case of a patient presenting an ascites, which revealed a non-Hodgkin lymphoma and chronic hepatitis B.     

Une métastase vésicale révélant une linite gastrique : à propos d’un cas et revue de la littérature

Secondary tumors of the bladder are rare, they represent less than 2% of all bladder neoplasms [1]. Most reported cases conform to autopsy series [2].Frequent primitives are the colon, prostate, rectum and cervix. In such cases, the bladder is affected by direct extension, in particular in advanced stage with peritoneal scattering. The vesical metastases of the gastric cancers are secondary to hematogenous spread of tumor cells. Brought back in the literature by some sporadic cases, there were seven cases of adenocarcinoma with cells signet ring [1]. We report the eighth case of bladder metastasis of gastric linitis which has the distinction to be revealing.     

Acanthosis nigricans malin révélant un cholangiocarcinome

IntroductionAcanthosis nigricans is a dermatosis characterized by the presence of a hyperpigmented, velvety cutaneous thickening in the flexural areas, especially axillary and inguinal fossas, and lateral faces of the neck. AN is usually a benign condition but can sometimes reveal an internal malignancy corresponds to a cutaneous paraneoplasic syndrome. Literature shows a predominant association with gastric adenocarcinoma. Here, we report a rare association between AN and cholangiocarcinoma.Case reportWe report a 43-year-old woman who presented an extensive AN associated to a tripe palms syndrome and florid cutaneous papillomatosis. She consulted in dermatology because of the itchiness of the lesions as well as for esthetics reasons. Complementary investigations enabled to diagnose a cholangiocarcinoma without visceral metastasis and she was treated by tumor resection and chemotherapy. Consequently, a slight improvement of the skin condition and the disappearance of pruritus were observed.ConclusionAN should be considered as cutaneous sign either of malignancy or endocrinopathy and therefore requires further investigations. The existence of extensive lesions, pruritus, tripe palms syndrome, florid cutaneous papillomatosis or mucous lesions, associated to an AN is a sign of malignancy should be investigated urgently the early diagnosis of which can lead to a better prognosis.     

Méningite récidivante révélant un syndrome de Bing-Neel

IntroductionBing-Neel syndrome is a rare complication of Waldenström macroglobulinemia, defined by monoclonal lymphoplasmocytic cells in the cerebrospinal fluid or in central nervous system biopsy.Case reportWe report a 47-year-old man, with no prior history, who presented a recurrent aseptic lymphocytic meningitis with central nervous manifestations. The presence of a monoclonal lymphoplasmacytic proliferation in cerebrospinal fluid, blood and bone marrow biopsy results was compatible with a diagnosis of Bing-Neel syndrome. Despite the absence of any specific treatment, there was no recurrence of symptoms at 4-month follow-up, and the MRI lesions remained stable.ConclusionWe report a case of Bing-Neel syndrome revealed by a recurrent meningitis. Outcome without treatment was favorable at 4-month follow-up.     

Une coagulopathie intravasculaire disséminée liée à une malformation veineuse

IntroductionCoagulopathy related to venous malformations can be life threatening. This complication is little known and underestimated.Case reportWe report the case of a 52-year-old female patient who presented with a left femoral fracture. She had a pre-existing muscular infiltrating venous malformation of the left hip. During surgery, she developed acute disseminated intravascular coagulation. The latter was probably a consequence of both surgery and localized intravascular coagulation that was unknown before the trauma.ConclusionIt is important to diagnose localized intravascular coagulation in venous malformations, since the risk of disseminated intravascular coagulation can be prevented by anticoagulant therapy.     

Bolus de méthylprednisolone révélant une hépatite auto-immune chez une patiente atteinte de sclérose en plaques

Background

Intravenous methylprednisolone pulse therapy is the standard treatment for relapsing multiple sclerosis. Only three reports have described the occurrence of autoimmune hepatitis after intravenous methylprednisolone pulse therapy for multiple sclerosis. We report herein the fourth published case of methylprednisolone-induced autoimmune hepatitis.

Exegesis

A Tunisian, 37-year-old woman with multiple sclerosis was treated with pulsed methylprednisolone. She did not have any history of liver disease; alcohol abuse or illicit drug use and liver tests were normal prior to treatment. Three weeks after the administration of intravenous methylprednisolone therapy, she presented with a profound elevation of liver transaminases. The viral serologies for hepatitis A, B and C, cytomegalovirus, Epstein-Barr virus and herpes simplex virus were negative. The antinuclear antibody and anti-smooth muscle antibody were positive at a titer of 1/100. Liver biopsy revealed portal lymphocytic infiltrate, interface hepatitis and fibrosis. According to the criteria of autoimmune hepatitis, the patient had a score of 16, indicating definite autoimmune hepatitis. Treatment with oral prednisone and azathioprine was initiated. Liver tests rapidly normalized.

Conclusion

To the best of our knowledge, this is the fourth case of autoimmune hepatitis developed after pulsed methylprednisolone for multiple sclerosis. Administration of corticosteroids and azathioprine after pulse methylprednisolone might be effective for preventing the development of autoimmune hepatitis.     

Résistance aux antivitamines K révélant une interaction avec la lécithine de soja

Vitamin K antagonists (VKA) are difficult to use because of a narrow therapeutic index and of a marked inter- and intra-individual variability among patients in the required dosage. This drug may interact with many other drugs and same with certain food compounds. We report the case of potential interaction between soy lecithin and Vitamin K antagonists in a 46 years-old woman. Subtherapeutic INR values were detected despite the increase gradually in dose and replacing acenocoumarol by fluindione. An enquiry of pharmacovigilance was conducted found the consumption of soy lecithin capsules. Fifteen days after its stopping, the INR values have really increased. Clinicians should think to the possibility of interaction between oral anticoagulants and food supplement that is increasingly used.     

Maladie cœliaque révélée par une pseudo-obstruction intestinale chronique

Introduction

Chronic intestinal pseudoobstruction is a rare and severe disease in which the symptoms and signs of intestinal obstruction occur without any mechanical cause. It can be primary (idiopathic) or secondary. It is a rare complication of coeliac disease. We report a case of a 36-year-old woman who was admitted in surgery department for recurrent occlusive syndrome which resolves after gastric aspiration. She underwent surgery for sigmoid volvulus. A chronic intestinal pseudo-obstruction was suspected. Clinical examination was normal. Anaemia (iron deficiency) was found without other signs of malabsorption. Oeso-gastro-duodenal fibroscopy was normal, but the jejunoscopy and the biopsy showed a subtotal villous atrophy of the jejunum. The coeliac serology was positive. Colonoscopy and biopsy were normal. A gluten-free diet was prescribed to the patient, with an improvement of the anaemia and relief of symptoms.

Conclusion

Coeliac disease is a rare etiology of secondary chronic intestinal pseudo-obstruction. It must be suspected in children and young adults.