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Objectives: We present a case report of Rosai-Dorfman disease with multiple extranodal involvement and favorable outcome after steroid therapy., Material and Methods: Rosai-Dorfman disease was diagnosed in a 17-year-old man with orbital, lacrymal, and rhinopharyngeal localizations. Histopathologic examination of lymphadenopathy biopsy revealed the diagnostic of Rosai-Dorfman disease. We present the histological findings leading to diagnosis and radiological data., Results: Conservative treatment with oral steroids resulted in the resolution of cervical lymphadenopathy and extranodal lesions. The patient was free of problems nine mouths after discontinuation of his treatment, demonstrating its efficacy and safety., Conclusion: Rosai-Dorfman disease is a benign disorder and steroid treatment must be initiated in cases with significant cosmetic deformity.

Extramedullary plasmocytoma (EMP) is a rare cell neoplasm most frequently localised in the upper respiratory tract. We report the case of a 43 year-old-man, with an unusual presentation of EMP developing in the mediastinum, two years after a diagnosis of solitary plasmocytoma of the bone which was successfully treated by local irradiation. In this aggressive presentation, we decided to perform an autologous hematopoietic stem cell transplantation. Two months after transplantation, CT scan showed disappearance of the mediastinal mass and immunofixation of the serum was normal. Selected cases of diffuse EMP, could benefit from intensive treatment followed by autologous hematopoietic stem cell transplantation.

Pituitary-hypothalamic axis Langerhans cell histiocytosis is an uncommon entity. It is a rare disease in adults. The CT and MR study provides us the best anatomo-topographic evaluation and determine the precise size of the lesion which are necessary to the treatment. We report a case of hypothalamic involvement by Langerhans cell histiocytosis accompanied by lesions in bone affecting a 31-year-old woman. The clinical, histiotological and CT/MR findings of histiocytosis X are described in this article.

A 29-year-old man with a 5-year history of Behçet's disease was admitted for fever, dyspnae, chest pain, and hemoptysis. A diagnosis of right ventricle and atrial thrombosis associated with a pulmonary artery aneurysm was made. The patient was treated with anticoagulants and prednisone. Since hemoptysis persisted, surgical excision of the intracardiac thrombosis was performed and histological findings were consistent with organizing thrombus and endomyocardial fibrosis. Transesophageal echocardiography 6 months later showed recurrence of the right ventricle thrombosis. A course of 6-monthly boluses of intravenous cyclophosphamide was begun. Currently, at 2 years of follow-up, the patient is asymptomatic.

Esophageal involvement in Behcet's disease is very rare, and normally is observed as aphtosis and esophagitis, but serious complications such as erosions, perforations, and stenosis may occur. We carried out this prospective study to evaluate the prevalence of esophageal involvement in Behcet's disease and to establish if routine endoscopy and/or manometry are necessary. Twenty-three patients who fulfilled the diagnostic criteria of the international study group for Behcet's disease were enrolled. None were taking drugs or had disease that might produce esophageal abnormalities or alter any existing changes due to the Behcet's disease itself. Twenty- three patients underwent esophagogastroduodenoscopy by a single observer. Esophageal biopsies were performed in thirteen patients and esophageal manometry in twenty. At the beginning of the study, the disease activity was defined by the presence of more than one symptom related to Behcet's disease, and upon the classification of Behcet's disease. Of the twenty three patients enrolled two were excluded from final analysis because of the presence of hiatus hernia. Thus, 13 men and 8 women, ranging in age from 20 to 63 years with a mean age of 36.2 years were included. Ten patients (47.6%) had active disease and four (19%) complained of upper gastrointestinal symptoms at the time of the study. Fourteen patients had endoscopic, manometric and/or microscopic abnormalities. Esophageal manometry was performed in twenty patients and was abnormal in 7 cases (35%). Esophageal biopsies were done in 13 patients and revealed histopathological abnormalities in 5 cases. Microscopic findings showed vasculities in one case, and non-specific inflammatory infiltration mainly consisting of neutrophils in 4 cases. Our results suggest that the prevalence of esophageal involvement in Behcet's disease is rather high and occur even in asymptomatic patients, but that this usually does not result in specific abnormalities.

Introduction: The occurrence of skin damage during systemic amyloidosis is common, but the appearance of bullous lesions is rare. Only twenty-seven cases have been reported in the literature. We report our observation of bullous amyloidosis during progression of renal amyloidosis., Observation: A 61 year-old man, presented with white, soft, palpebral edemas of the lower limbs, without scutulum involvement, associated with a large cubital nerve that had appeared in March 1997. Biological explorations revealed a nephrotic syndrome. Pathologic study of the renal biopsy concluded in amyloidosis. Treatment with colchinine stabilized the renal damage. One year later, a non-pruriginous, papular and bullous eruption occurred, localized essentially in the axillary and inguinal-crural folds of the forearms and legs. In the presence of an amyloidal deposit and intra-epidermal detachment, the cutaneous biopsy was evocative of bullous amyloidosis. The search for concomitant myeloma was negative. Treatment with colchinine was effective. The bullous lesions disappeared after 2 months, and 21 months later, renal damage was still stable., Discussion: These particularities in evolution are exceptional and have never been described. A hypothetical modification in the physico-chemical properties of the amyloidal protein might explain the bullous eruption and stabilization of renal damage.

Introduction: The association of Behçet's disease and of Crohn's disease is exceptional, and raises a nosological problem., Case Report: A 24 year-old female developed since 1996 chronic diarrhea, abdominal pain, recurrent oral and genital ulcer and polyarthralgia. Endoscopic colonoscopy examination showed a diffuse colitis. Histological examination revealed epitheloid granuloma without vasculitis compatible with a Crohn's disease. The patient was treated with oral prednisone (1mg/kg/day). In November 1998, she was admitted for diarrhea. Many pseudofolliculitis lesions, uveitis and positive pathergy test were noted. The HLA was B 51. The diagnosis of Crohn's disease associated with Behçet's disease was made. She was treated with high doses of prednisone (1mg/kg/day and 6 monthly intravenous pulses of cyclophosphamide). Skin lesions and diarrhea improved within few days, and cleared completely within five months. Presently, the patient remains clinically free of disease., Conclusion: While being of different pathogenic origin, Behçet's disease and Crohn's disease may coexist within one and the same patient and cause diagnostic and therapeutic problems.

The association of Behçet's disease and non-Hodgkin's lymphoma is very rare. The first case of primary intestinal lymphoma with intestinal Behçet's disease is reported. A 37 year-old woman had been treated for Behçet's disease with colchicine for 2 years. In July 1997, she developed uveitis and was treated by monthly intravenous pulses of cyclophosphamide for 6 months, but uveitis persisted; so cyclophosphamide was replaced by cyclosporin 300 mg/day. One month later, she suffered from diarrhea. Colonoscopy showed ileocoecal ulcerations. Histological examination of surgical biopsy revealed B large cell type lympocytic malignant lymphoma and vasculitis lesions compatible with intestinal Behçet's disease. Cyclosporin was stopped and treatment with prednisone was instituted. The relationship between non-Hodgkin's lymphoma and immunosuppressive drugs in Behçet's disease is discussed.

Objective: We aimed to describe the epidemiological and clinical aspects of deep vein thrombosis (DVT) in Behçet's disease (BD) and to determine the patients at high risk for this complication., Methods: Among 113 patients with BD according to the international criteria for classification of BD, those with DVT were retrospectively studied. The diagnosis of DVT was made in all cases using conventional venous angiography, venous ultrasonography and/or thoracic or abdominal computed tomography. Patients were divided in two subgroups according to the occurrence of DVT other than cerebral thromboses. The medical records of these patients were reviewed in order to investigate their past medical history and evaluate their response to the treatment prescribed. Clinical and genetic factors (HLA B51 and MICA 6) that might contribute to DVT were analysed by comparing patients with and without DVT. Results of our series were compared to those of other series in the literature. Statistical analysis was by Chi square with necessary correction and Fischer tests., Results: Forty-four patients (38.9%) had deep vein thrombosis of various systems with 81 localisations. There were 40 men and four women (mean age 28.1 years; range 17-60). DVT appeared after the onset of disease with a mean delay of 3.8 years. In 6 cases, DVT revealed BD. When we evaluated the risk of DVT coexistence with other clinical findings and genetic factors (HLA B51 and MICA 6), we found a significant positive correlation with sex, and positive pathergy test., Conclusion: In our series, occurrence of DVT was significantly associated with male gender and positive pathergy test.

Various clinical and immunological extrahepatic manifestations were described during infection by hepatitis C virus (HCV). We reported a rare association, within the same patient, made up with a cutaneous leucytoclastic vasculitis, cryoglobulinemia and aplastic anemia. A 70 years old woman with infection by HCV diagnosed four years ago, was hospitalized due to upper members purpura and six cutaneous ulcerations. Complete blood count revealed a pancytopenia. Prothrombin time was at 65%, AST, ALT an d GT were within normal limits. Test for antibodies to HCV en serum was positive. Bone marrow aspiration showed marked hypocellularity. A skin biopsy showed leucocytoclastic vasculitis of small vessels. Interferon a therapy was not indicated because aplastic anemia. Simultaneous occurrence of cutaneous vasculitis, cryoglobulinemia and aplastic anemia during HCV infection lays the question of their mechanism. Purpura and cutaneous ulcerations can be due to cryoglobulinia.

Introduction: Vertebral involvement of actinomycosis is extremely rare and associated spinal cord compression is unusual., Exegesis: We report a case of a 31-year-old man with vertebral actinomycosis presenting with spinal cord compression. Magnetic resonance imaging demonstrated a paravertebral abscess and lytic areas on the vertebral body of C5 requiring emergency surgery and antibiotic treatment. The patient was still asymptomatic after 12 months of follow-up., Conclusion: Clinical aspects of this unusual localization are reviewed. Treatment may be particularly difficult.

Introduction: Langerhans' cell histiocytosis is a rare disorder of unknown etiology characterized by a wide clinical spectrum and varied behavior. Diabetes insipidus is a relatively common feature in Langerhans' cell histiocytosis. The presence of both diabetes insipidus and mellitus associated with histiocytosis in an adult is rare. To our knowledge, only three previous cases have been reported., Exegesis: We report the clinical presentation, pathologic findings and clinical progress in an adult female who had disseminated Langerhans' cell histiocytosis (hypothalamic infiltration, multifocal bone involvement) associated with both diabetes insipidus and mellitus., Conclusion: The pathogenesis of diabetes mellitus in such an association will be discussed.

Objective: To assess prolactin level and its possible role in the pathogenesis and disease expression of patients with Behçet's disease (BD)., Materials and Methods: Twenty-eight patients with the diagnosis of Behçet's disease were selected. They all fulfilled the international criteria for BD. The control group consisted of 17 males and 6 females. Patients are divided into subgroups according to the clinical and paraclinical characteristics such as disease duration, ocular, articular and neurological involvement. Serum prolactin was measured by ELISA using Merck Kits on a Maggia 7000 Analyser. The normal range in males was<17ng/ml in males and<18ng/ml in females. The mean PRL level in each group and subgroup was calculated and compared with Student's t test., Results: The mean prolactin level in the BD group (mean=13.76, SD=6.82), was higher than in the control group (mean=10.13, SD=5.46) with no statistically significant difference. The mean prolactin levels in all subgroups of patients with BD were higher than normal, but no statistically significant difference was shown between these subgroups., Conclusion: Hyperprolactinemia occurred in a small number of patients with BD and its significance remained unclear. Serum PRL level did not correlate with disease manifestations and activity.

Introduction: Thiamine deficiency can be determined by various clinical signs; some of these symptoms may be acute, and require an urgent diagnosis. In countries such as ours with a high standard of living, this disorder is more commonly observed in cases of severe alcoholism, and cases of diet-associated thiamine deficiency are rare, and therefore not easily recognized. The metabolic disorders resulting from vitamin B1 deficiency are responsible for the well-known central or peripheral neurological symptoms, and also for the less common and often more acute cardiovascular reactions. Immediate thiamine/vitamin B1 supplementation is of major importance. The rapid reversal of symptoms following this treatment is often considered as a diagnostic index., Exegesis: In this study, an original case of diet-associated thiamine deficiency has been reported, with clinical symptoms including myocarditis and subacute peripheral nerve involvement in a young adult. The disorder was the result of a thiamine deficiency in the diet, which was exclusively based on milled rice., Conclusion: The present report is interesting both as regards its clinical aspects and its etiology, and it emphasizes the importance in the differential diagnosis of a given case of taking the possibility of diet-related thiamine deficiency into account, although this is an uncommon etiology in developed countries.

Aim: To study the clinical characteristics and the evolution of vena cava thrombosis (VCT) in Behçet's disease (BD), as well as their association with other severe symptoms., Patients and Methods: Among 121 BD, we selected those with VCT. All patients fulfilled the diagnostic criteria of the international study group of Behcet's disease. Different clinical and paraclinical parameters were determined and compared with the remaining group of patients (not having VCT) with chi 2 test with Yates' correction. Protein C, protein S and antithrombin III and anticardiolipin antibody (aCL) levels were measured in 9 patients; anti-beta 2-glycoprotein I antibodies (a beta 2GPI) were determined in 3 patients., Results: Ten patients had a vena cava thrombosis (8.2%). They were all male with an average age of 35 years (range: 30-42). We had 3 cases of superior vena cava thrombosis, 6 cases of inferior VCT, and one case of both. The average delay to diagnosis of the VCT from the date of the BD diagnosis was 4.5 years (range: 6 months-14 years), and in one case the thrombosis revealed the disease. All patients were clinically symptomatic and the installation of the symptoms were progressive and insidious in all cases. Six patients had Budd-Chiari syndrome and 4 had a phlebitis of a lower limb. Among all the clinical characteristics studied, only neurological manifestations was significantly higher in patients with VCT (p = 0.001). Protein C, protein S and antithrombin III levels were normal in all cases. One patients was positive for IgG aCL and no patient was positive for a beta 2GPI. All our patients were treated by anticoagulation therapy and high-dose prednisone combined with intravenous cyclophosphamide in 5 cases. One patient died due to liver failure. The 9 others are clinically improved (6 cases) or stable (3 cases) after an average 2.5 year course.

Introduction: It is uncommon that lymph node enlargement is diagnostic of systemic amyloidosis as found in the case reported in this study., Exegesis: This study examined the case of a 49-year old male with chronic bronchitis in whom in 1990 the presence had been detected of an isolated cervical lymphadenopathy, 2 cm in diameter, and which had previously remained unnoticed. In 1993, a significant number of other peripheral adenopathies also appeared in various locations, i.e., cervical, axillary, inguinal. Chest and abdominal CT-scans revealed several mediastinal and abdominal lymphadenopathies. The histological study with Congo red stain of a cervical lymph node biopsy determined the diagnosis of amyloidosis. The patient was at that time asymptomatic. In September 1997, upon physical examination the following were found: lower limb edema, superior vena cava syndrome, and several cervical lymphadenopathies. Abdominal ultrasonography showed enlarged kidneys, and homogeneous splenomegaly. Biological examination determined the existence of a nephrotic syndrome with renal failure and creatinemia of 350 mumol/L. Due to superior vena cava syndrome worsening, cervical lymph node removal was performed. However, the patient died after rapid renal failure., Conclusion: Although it is a rare occurrence, amyloidosis should be taken into consideration in the differential diagnosis of isolated lymphadenopathy. Congo red stain amongst others, and an immunohistochemical study should be performed in cases of uncertain diagnosis.

Eight patients presented neurological signs secondary to Brucella infection. The clinical presentation was a meningoencephalitis in three cases, a meningoencephalomyelitis in one case, an epiduritis with spinal cord compression in one case, an acute polyradiculoneuritis in two cases and a chronic polyradiculoneuritis in one case. Acoustic nerve was impaired in seven cases. Cerebrospinal fluid (CSF) analysis revealed a lymphocytic meningitis and a high protein concentration in all cases. The agglutination test titers were elevated in the serum and in the CSF of seven patients (> or = 1/80) and two patients respectively. Brucella melitensis culture was disclosed in the blood of one patient and in the CSF of two patients. Three patients were treated by the association cycline and rifampicin whereas a tritherapy including cycline, rifampicin and TMP-SMZ was used in the other cases. Outcome was favorable in seven cases. This study outlines the polymorphism of neurological manifestations due to brucellosis, even in familial cases and this diagnostic must be especially done in Middle East and South Mediterranean countries.