[Vascular manifestations of Behcet's disease]. / Les complications vasculaires de la maladie de Behçet.

Behçet disease is a multi-systemic complex vasculitis with unknown etiology characterized by different clinical involvements, including mucocutaneous, ocular, vascular, articular, neurological and gastrointestinal manifestations. Growing evidence supports that different phenotypes, characterized by clusters of co-existing involvements, can be distinguished. Namely, the vascular phenotype identifies a specific group of patients who suffer from recurrent inflammatory thrombosis and arterial involvement. Vascular disease develops in up to 40% with a definite male preponderance and is usually an early manifestation. It is one of the main causes of death in Behçet's disease. Venous involvement is significantly more common than arterial disease and lower extremity deep vein thrombosis is its most frequent manifestation. Arterial disease involves mostly pulmonary arteries and aorta and manifests mainly in the form of aneurysms. Glucocorticoids and immunosuppressant's are the recommended first-line treatments in vasculo-Behçet. Furthermore, randomized controlled trials are still needed to assess the role of adding anticoagulation to current standard therapy in venous thrombosis in Behçet's disease and to assess the role of anti-TNF alpha therapy in vasculo-Behçet.

Venous thromboembolism and hyperhomocysteinemia as first manifestation of pernicious anemia: a case series.

BACKGROUND: Hyperhomocysteinemia has been suspected of favoring thrombosis. Several case-control studies and even a meta-analysis have confirmed a link between venous thrombosis and hyperhomocysteinemia. Homocysteine is due to genetic and acquired factors (poor diet in folate and vitamin B12, older age, renal impairment, thyroid diseases, and malignancies) induced by the intake and the concentrations of vitamin B9 or B12 in the majority of cases. CASES PRESENTATION: We report the cases of four Moroccan patients who presented with acute vein thrombosis of different sites: a 34-year-old man, a 60-year-old man, a 58-year-old man, and a 47-year-old woman. All patients had a low level of cobalamin with marked hyperhomocysteinemia with normal serum and red cell folic acid. Venous thrombosis revealed pernicious anemia in all patients. Their low levels of cobalamin, atrophic gastritis, and positive results for gastric parietal cell antibodies confirmed the diagnosis of pernicious anemia. There was no evidence of immobilization, recent surgery, malignancy, antiphospholipid antibody, myeloproliferative disorder, or hormone replacement therapy. No deficiencies in protein C and protein S were detected; they had normal antithrombin III function and factor V Leiden; no prothrombin gene mutations were detected. Treatment included orally administered anticoagulation therapy and cobalamin supplementation. The outcome was favorable in all cases. CONCLUSIONS: These reports demonstrate that pernicious anemia, on its own, can lead to hyperhomocysteinemia that is significant enough to lead to thrombosis. Understanding the molecular pathogenesis of the development of thrombosis in patients with hyperhomocysteinemia related to Biermer disease would help us to identify patients at risk and to treat them accordingly. The literature concerning the relationship between homocysteine and venous thrombosis is briefly reviewed.

Kikuchi-Fujimoto disease: report of 4 cases and review of the literature.

INTRODUCTION: Kikuchi-Fujimoto disease or histiocytic necrotizing lymphadenitis is a benign disease of unknown origin predominantly affecting young women and presenting in the form of cervical lymphadenopathy and/or prolonged fever. MATERIAL AND METHODS: The authors report 4 cases of Kikuchi-Fujimoto disease diagnosed in the Internal Medicine Department of Ibn Sina university hospital in Rabat between 2009 and 2010. RESULTS: These 4 women with a mean age of 27±8.6years [16-37] were admitted with febrile syndrome and cervical lymphadenopathy. The diagnosis was based on histological examination of a lymph node biopsy. The disease was associated with systemic lupus erythematosus in one case and actinomycosis in another case. A favourable course was observed in response to corticosteroid therapy in two patients, antibiotic therapy in one patient and antipyretic treatment alone in the fourth patient. CONCLUSION: In the light of these four cases, the authors discuss the diagnostic difficulties, the modalities of treatment of Kikuchi-Fujimoto disease and its clinical course.

[Kikuchi-Fujimoto's disease: Report of 4 cases and literature review]. / Maladie de Kikuchi-Fujimoto : à propos de quatre cas et revue de littérature.

INTRODUCTION: Kikuchi-Fujimoto's disease or histiocytic necrotizing lymphadenitis is a benign disease predominantly occurring in young women which etiology remains unknown and revealed by cervical lymphadenitis and/or prolonged fever. OBSERVATIONS: This report describes a survey of four patients who developed Kikuchi's lymphadenitis occurring concomitantly with LES in one case and actinomycosis in another case. The definite diagnosis is usually made through histopathological examination of a lymph node biopsy. The evolution was favorable under corticosteroid therapy in two patients, antibiotics in the third and only antipyretic in the fourth. CONCLUSION: The authors bring report through these three observations, the diagnostic difficulties, the therapeutic means of the disease of Kikuchi, as well as its evolutionary aspects.

[Ocular complications of HIV infection: experience of the Northern Excellence Pole of Morocco]. / Les complications oculaires au cours de l'infection par le VIH : expérience du pôle d'excellence Nord du Maroc.

INTRODUCTION: HIV infection is associated with a wide variety of ophthalmic manifestations. The objective of this study was to identify the ocular complications of HIV/AIDS in Morocco. MATERIAL AND METHODS: A retrospective study conducted in the internal medicine department of the Rabat Teaching Hospital between 1998 and 2008. All HIV-positive patients were retained for the study. Each patient had an exhaustive ocular examination. RESULTS: Of 115 ophthalmologic examinations, 28 were abnormal. Twenty-seven patients had stage C HIV infection and one patient was in stage A. The ocular manifestations were: 21 cases of ocular HIV-related anomalies (microangiopathies), four cases of cytomegalovirus (CMV) retinitis, one case of toxoplasmosis chorioretinitis, one case of CMV retinitis associated with toxoplasmosis chorioretinitis, two cases of Cryptococcus neoformans infection, two cases of varicella-zoster virus (VZV) retinitis, and one case of conjunctival Kaposi sarcoma. The CD4 count average was of 86±91/mm(3). Twenty-six patients had a CD4 count lower than 200/mm(3). Progression was favorable in 85 % of the cases, with three cases of blindness: bilateral in a case of VZV retinitis and unilateral in two cases of CMV retinitis and toxoplasmosis chorioretinitis. CONCLUSION: CMV infection is the main ocular opportunistic infection in our series. However, Highly Active Antiretroviral Treatment (HAART) may be the cause for the decline in the prevalence of ocular diseases and visual impairment in HIV/AIDS.

[Limb arteritis in Horton disease: five cases]. / Atteinte des artères des membres et maladie de Horton: à propos de cinq cas.

INTRODUCTION: Giant cell arteritis of the limbs is rarely reported. It may be underestimated because it is usually asymptomatic. The aim of this study was to describe the distinctive features of this involvement. METHODS: Retrospective study of 50 patients with giant cell arteritis diagnosed from January 1985 to December 2007, satisfying the American college of rheumatology (ACR) classification criteria. All five patients had typical lesion at examination of temporal artery biopsy. Among these patients, those with ischemic manifestations of the limbs were selected. All patients disclosed elevated acute phase reactants and typical radiological findings. Temporal artery histopathology was made necessary to select patients. RESULTS: Five female (mean age: 66.8 years) out of 50 patients (10%) with temporal arteritis presented with symptomatic limb arteriopathy. Limb arteritis was the presenting feature in four patients. Clinical presentation was similar to other non-inflammatory arteritis. One patient presented with involvement of her four limbs. Typical arteriographic abnormalities were presents in all patients (occlusion or moniliform stenosis without atheromatous lesions). Despite therapy with corticosteroids associated to anticoagulants or antiplatelet drugs, four amputations were necessary. CONCLUSION: Specific limb involvement may be the presenting feature of temporal arteritis. It can mimic arteriosclerosis vascular disease, particularly prevalent in this age group.

Systemic lupus erythematosus and multiple myeloma: an uncommon association. Two cases and literature review.

The association of systemic lupus erythematosus and multiple myeloma is uncommon. We report two cases of systemic lupus erythematosus associated to multiple myeloma. The cases are discussed in the light of a review of the literature. The clinical, laboratory and radiographic findings of the patients, as well as the subsequent therapeutic approach are discussed. A systematic review of all the other cases of this association is performed. We report two female patients of 50 and 35 years old who developed a multiple myeloma seven and three years respectively after the diagnosis of systemic lupus erythematosus. In the second case, systemic lupus erythematosus was associated to monoclonal gammopathy. One patient died after three months and one patient is still in remission after three years of the diagnosis of multiple myeloma. The coexistence of systemic lupus erythematosus and multiple myeloma is very rare and the possible pathogenetic mechanisms underlying this association remain unclear.

[Prolonged fever of unknown origin in Behçet's disease. Pay attention to vessels!]. / Fièvre prolongée inexpliquée au cours de la maladie de Behçet. Attention aux vaisseaux!

Behçet's disease is a systemic vasculitis characterized by the association of recurrent oral and genital ulcers to systemic involvements, particularly ocular, nervous and vascular manifestations. Contrary to other vasculitis, prolonged fever of unknown origin is rare in Behçet's disease. We report a case of a 26-year-old man presenting prolonged fever for two months. Physical examination showed oral, genital ulcers and pseudofolliculitis. The sedimentation rate was increased. Chest and abdominal computed tomography revealed thrombus in the inferior vena cava and portal vena. Outcome was favorable with glucocorticoid and anticoagulant therapy. Prolonged fever occurring during Behçet's disease should prompt a search for a vascular injury.

Contribution of spinal MRI for unsuspected cobalamin deficiency in isolated sub-acute combined degeneration.

Sub-acute combined degeneration (SCD) is a rare cause of demyelination of the dorsal and lateral columns of the spinal cord and is a neurological complication due to vitamin B12 deficiency. Revealing forms of SCD without anemia are rare. We report a case of SCD of the spinal cord in a 33-year-old woman without anemia but with a 10-month history of paresthesis and urine imperiosity. Magnetic resonance imaging (MRI) of the spine showed intramedullary hyperintensity seen on T2-weighted images in the posterior column of the cervico-dorsal spinal cord, extending from C1 to D1. A diagnosis of SCD of the spinal cord was considered and confirmed by a low serum cobalamin. The patient was treated with vitamin B12 supplements and showed gradual improvement in her clinical symptoms.

Pernicious anemia in a young man with systemic lupus erythematosus.

Association of pernicious anemia and systemic lupus erythematosus is rare, although both diseases are autoimmune origin. We describe the case of a 40-year old man with systemic lupus erythematosus (SLE) who developed pernicious anemia. The cobalamin deficiency was revealed by macrocytic pancytopenia. After 1 month of vitamin B12 treatment, hemoglobin and white blood cell count remain normal but thrombocytopenia persists and was considered as immunologic from SLE origin requiring corticosteroids.

Fever revealing Behçet's disease: Two new cases.

Behçet's disease (BD) is an uncommon cause of fever of unknown origin. We report two cases, both involving 42-year-old males, who initially presented with prolonged fever and who were ultimately diagnosed as having BD after a delay of 12 and 21 months, respectively. Both patients developed pulmonary aneurysms. Although fevers resolved after therapy, both patients died within the first year after diagnosis. Clinicians should be aware that long-term fever may be an inaugural sign of BD, especially in individuals living in countries along the ancient Silk Road or Mediterranean basin.

Kaposi's sarcoma in association with Behcet's disease: case report and literature review.

OBJECTIVES: To describe a case of Behcet's uveitis associated with Kaposi's sarcoma occurring simultaneously in a patient and to review the literature on iatrogenic Kaposi's sarcoma. METHODS: We describe the case of a 44-year-old Moroccan man, who developed a Kaposi's sarcoma 8 months after immunosuppressive therapy for ocular Behçet's disease. He was treated with corticosteroids and cyclophosphamide (Exdoxan, Baxter) pulse for 6 months followed by oral azathioprine (Imurel, Glaxo Smith Kline). Literature searches were performed on iatrogenic Kaposi's sarcoma and other cases of such association and the potential pathogenic mechanisms involved. RESULTS: Iatrogenic Kaposi's sarcoma is widely reported to develop after renal transplantation during immunosuppressive therapy. Less commonly, Kaposi's sarcoma occurs in patients receiving long-term corticosteroids or immunosuppressive therapy for rheumatic diseases. It is considered to be induced by activation of latent human herpes virus 8. To our knowledge, this is the second reported case of iatrogenic Kaposi's sarcoma in a patient with ocular Behçet's disease. Interferon-alpha is of value for patients with both conditions. CONCLUSION: This case report underscores the relationship between environmental and infectious factors, drug-induced immunosuppression, and the development of Kaposi's sarcoma.

[Primary Gougerot-Sjogren's syndrome presenting as a cerebellar syndrome]. / Syndrome cérébelleux isolé révélant un syndrome de Gougerot-Sjögren primaire.

Primary Sjogren's syndrome is an autoimmune exocrinopathy with various extraglandular complications. Central neurological manifestations were described in 20 percent of cases. Cerebellar syndrome is uncommon in Sjogren's syndrome, but can occur after diagnosis, or rarely as an inaugural sign of the disease. We report the case of a 54-year-old man who presented a cerebellar syndrome with xerostomy and xerophthalmos. The diagnosis of primary Sjorgren's syndrome was confirmed on the accessory salivary gland biopsy. The patient received oral and intravenous corticosteroid therapy associated with cyclophosphamide monthly. Outcome was good.

[Neurological manifestations of vitamin B12 deficiency: a retrospective study of 26 cases]. / Les troubles neurologiques par carence en vitamine B12: étude rétrospective de 26 cas.

PURPOSE: Neurological symptoms of B12 vitamin deficiency are polymorph. Causes are dominated by Biermer's disease and B12 vitamin non dissociation that is frequent in the elderly. METHODS: We realized a retrospective study during 11 years. Patients with neurological symptoms associated to megaloblastosis were included. Treatment with B12 vitamin lead to haematological manifestation regression. RESULTS: 26 cases were analyzed. Mean age was 50+/-14.5 years and there were 11 women and 15 men. Neurological signs included combined medullar sclerosis (N = 10), peripheral neuropathy (N = 10), isolated paresthesia (N = 5) and inferior limb pyramidal syndrome (N = 1). Neurological signs revealed vitamin B12 deficiency in 4 cases. Mean haemoglobin rate was 6.2+/-2.6 g/dl, mean MCV was 109+/-56 fl. Eight patients had macrocytic anaemia, nine bi-cytopenia and eight deep pancytopenia. Electromyography (N = 8) confirmed neuropathy and medullar MRI (N = 2) showed antero-posterior cordonal demyelinisation of cervico-dorsal medulla. Causes of B12 vitamin deficiency were Biermer's disease (N = 11), non dissociation of B12 vitamin's syndrome (N = 8) and partial gastrectomy (N = 1). In 6 patients, no aetiology was found. Treatment with parenteral vitamin B12 induced neurological symptoms regression in 14 cases. Patient with neurological disorder had significantly higher platelet count and haemoglobin level as compared with patients without neurological disorder. Reticulocyte crisis was more precocious in patients with neurological disorders. CONCLUSION: Neurological symptoms in vitamin B12 deficiency are frequent. We insist on isolated forms, inaugural forms and on the interest of medullar MRI for early diagnosis.