[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.

[COVID-19: Coagulopathy and thrombosis]. / COVID-19 : coagulopathie et thrombose.

The SARS-CoV-2 virus caused a global pandemic within weeks. Many patients with severe COVID-19 present with coagulation abnormalities, including increase D-dimers. This coagulopathy is associated with an increased risk of death. Furthermore, a substantial proportion of patients with severe COVID-19 develop sometimes unrecognized, venous thromboembolic complications. A better understanding of COVID-19 pathophysiology, in particular hemostatic disorders, will help to choose appropriate treatment strategies. A rigorous thrombotic risk assessment and the implementation of a suitable anticoagulation strategy are required. We review here the characteristics of COVID-19 coagulation laboratory findings in affected patients, the incidence of thromboembolic events and their specificities, and potential therapeutic interventions.

Evaluation and management of thromboprophylaxis in Moroccan hospitals at national level: the Avail-MoNa study.

Venous thromboembolism (VTE) is a common clinical problem that is associated with substantial morbidity and mortality. The aim of this study was to describe the clinical practices in VTE prophylaxis in university and peripheral hospitals in Morocco. This is a national, cross-sectional, multicenter, observational study assessing the management of the VTE risk in selected Moroccan hospitals (four university and three peripheral). The thromboembolic risk of the selected patients was assessed according to the American College of Chest Physicians (ACCP) guidelines (2008). We hypothesized that interventions for VTE guideline implementation in those hospitals may improve prophylaxis use for hospitalized patients. A total of 1318 patients were analyzed: 467 (35.5%) medical and 851 (64.5%) surgical. The mean age of patients was 52.6 ± 16.5 years, and 52.7% were female. A total of 51.1% patients were considered to be at risk of VTE according to ACCP guidelines and were eligible for thromboprophylaxis (TP). Medical patients were more likely to present risk factors than surgical patients (53.6 vs. 50.7%, respectively). TP was prescribed for 53.1% of these patients, 57.4% in at-risk surgical patients and 50.3% in at-risk medical patients. TP was also prescribed for 42.9% of non-at-risk patients. The concordance between the recommended and the prescribed prophylaxis was poor for the total population (kappa = 0.110). TP did not improve sufficiently in our hospitals, even after implementation of the guidelines. New strategies are required to appropriately address TP in hospitalized patients.

A case report of rare palmoplantar keratosis and nail dystrophy with imatinib.

A 48-year-old woman developed palmoplantar hyperkeratosis during treatment with imatinib (400mg/day) for treatment of chronic myeloid leukemia. After 5months of treatment, she developed plantar lesions with yellow-brownish plaques and palmar desquamations. The skin biopsy has eliminated psoriasis. Imatinib was discontinued, and treatment with an emollient balm and a soothing repair cream with an improvement of symptoms. A French imputability assessment score of I3 was obtained, indicating a probable relationship between the side effect and imatinib. In our case, the skin adverse events require definitive drug discontinuation and change of treatment.

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.

[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.

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.

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.

[Pyoderma gangrenosum revealing Takayasu's arteritis]. / Pyoderma gangrenosum révélant une artérite de Takayasu.

BACKGROUND: Pyoderma gangrenosum is a form of neutrophilic dermatosis often associated with inflammatory disease or blood dyscrasias. We report a case of pyoderma gangrenosum revealing Takayasu's arteritis. OBSERVATION: A 26 year-old woman was followed since 2002 for pyoderma gangrenosum occurring during pregnancy and regressing under general corticosteroids. In January 2004, immediately after childbirth, she presented an extensive ulcerated lesion on the right forearm. Histological examination of a biopsy sample from this solution pointed towards pyoderma gangrenosum. Somatic examination showed reduced peripheral pulse on the right side, asymmetric blood pressure between the two arms and vascular murmur in the carotid, femoral and renal arteries. Based on laboratory and x-ray examinations, Takayasu's arteritis was diagnosed and chronic viral hepatitis B detected. Resumption of treatment with prednisone (1 mg/kg/d) resulted in resolution of the wounds within 1 month. DISCUSSION: Although pyoderma gangrenosum is a classic complication in Takayasu's arteritis in Japan, this combination is rare in Europe, North America and North Africa. This case was singular in terms of the occurrence of pyoderma gangrenosum with each pregnancy. However, a number of epidemiological and clinical features were consistent with those reported in the literature, namely presentation predominantly in female patients, young age and involvement of the limbs. In 60% of cases, pyoderma gangrenosum precedes Takayasu's arteritis. The recommended mode of treatment comprises oral corticosteroids as first-line therapy followed by cyclosporine as second-line treatment.

[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.

[Takayasu's disease in Morocco. Report of 47 cases]. / La maladie de Takayasu au Maroc. A propos de 47 observations.

PURPOSE: Takayasu's disease is a chronic inflammatory arteritis involving large vessels in young women. We studied Moroccan patients to evaluate clinical, biological, radiological and evolution features of this disease in our country. METHODS: Forty-seven patients with Takayasu's arteritis were studied retrospectively between 1988 et 1999. RESULTS: In our series involvement of the aortic arch and its branches was more frequent than the abdominal aorta and its branches. Stenotic lesions of renal arteries were rare. Ultrasound was useful in the diagnosis and the monitoring of the disease. Treatment with glucocorticoids gave good results, with improvement in half of the patients and remission with stabilisation in 40% of cases. Tuberculosis occurred in 8.5% of patients. CONCLUSION: The use of ultrasound and computed tomography angiography is helpful for the diagnosis and monitoring of the disease progression. Glucocorticoids help to induce long remission in about 80% of treated patients. We don't find any relationship between Takayasu's arteritis and tuberculosis.