K. Bouslama

La maladie de Behçet en Tunisie. Étude clinique de 519 cas

PURPOSE To describe clinical characteristics of Behçets disease in Tunisia. METHODS Its a retrospective and multicentric study conducted by the Tunisian society of internal medicine. Inclusion criteria were those of the international study group. Were also included patients without international study group criteria but with at least one manifestation among arthritis, venous thrombosis or neurological manifestation with oral and genital ulceration or oral ulceration and skin lesions. RESULTS Five hundred and nineteen patients were included. 87.5% of them fulfilled the international criteria. The male to female ratio was 2,7. The mean age was 28.7+/-9.3 years at onset and 32.7+/-9.2 years at diagnosis. The incidence of each manifestations was as follows: oral ulcers: 100%, genital ulcers: 87.5%, pseudo-folliculitis: 67.6%, erythema nodosum: 17.5%, positive pathergy test: 51%, joint involvement: 55%, uveitis: 32.2%, vein thrombosis: 24.9%, arterial aneurysms: 3.9%, neurological involvement: 11.6%. The frequency of HLA B51 antigen was 35% among the 187 patients tested. There was no difference in the manifestations of the disease between patients having B51 and those lacking it. Venous thrombosis (29.8 vs 11.4%), arterial involvement (4.4 vs 1.4%) and uveitis (37.5 vs 17,9%) were significantly more frequent in men whereas erythema nodosum (22.9% vs 15.6%) and joint involvement (70,7 vs 49.9%) more frequent in women. The mean follow up was 6,1+/-5.7 years. Mortality rate was 2.3% in our series. CONCLUSION Our study confirms the androtropism of the disease in Mediterranean and Middle east countries. Positive pathergy test and venous thrombosis were more frequent in our study, like those from Mediterranean region. Whereas, ocular and neurological involvement were quite less frequent in our series.

Article originalLa maladie de Behçet en Tunisie. Étude clinique de 519 casBehçet's disease in Tunisia. Clinical study of 519 cases

PURPOSE To describe clinical characteristics of Behçets disease in Tunisia. METHODS Its a retrospective and multicentric study conducted by the Tunisian society of internal medicine. Inclusion criteria were those of the international study group. Were also included patients without international study group criteria but with at least one manifestation among arthritis, venous thrombosis or neurological manifestation with oral and genital ulceration or oral ulceration and skin lesions. RESULTS Five hundred and nineteen patients were included. 87.5% of them fulfilled the international criteria. The male to female ratio was 2,7. The mean age was 28.7+/-9.3 years at onset and 32.7+/-9.2 years at diagnosis. The incidence of each manifestations was as follows: oral ulcers: 100%, genital ulcers: 87.5%, pseudo-folliculitis: 67.6%, erythema nodosum: 17.5%, positive pathergy test: 51%, joint involvement: 55%, uveitis: 32.2%, vein thrombosis: 24.9%, arterial aneurysms: 3.9%, neurological involvement: 11.6%. The frequency of HLA B51 antigen was 35% among the 187 patients tested. There was no difference in the manifestations of the disease between patients having B51 and those lacking it. Venous thrombosis (29.8 vs 11.4%), arterial involvement (4.4 vs 1.4%) and uveitis (37.5 vs 17,9%) were significantly more frequent in men whereas erythema nodosum (22.9% vs 15.6%) and joint involvement (70,7 vs 49.9%) more frequent in women. The mean follow up was 6,1+/-5.7 years. Mortality rate was 2.3% in our series. CONCLUSION Our study confirms the androtropism of the disease in Mediterranean and Middle east countries. Positive pathergy test and venous thrombosis were more frequent in our study, like those from Mediterranean region. Whereas, ocular and neurological involvement were quite less frequent in our series.

Pancréatite aiguë et syndrome hémophagocytaire au cours d'une poussée lupique : À propos d'une observation

Resume La pancreatite aigue et le syndrome hemophagocytaire (SH) sont rares au cours du lupus erythemateux systemique (LES). En revanche, l’association des 2 complications au cours d’une poussee lupique n’a jamais ete rapportee auparavant. Une jeune femme âgee de 31 ans, suivie pour LES depuis 7 ans a ete admise pour douleurs abdominales et fievre. Le diagnostic de pancreatite aigue a ete retenu devant une hyperamylasemie et l’augmentation du volume du pancreas avec collection intersplenorenale a la tomodensitometrie. Devant la pancytopenie, la cytolyse, la cholestase, l’hypertriglyceridemie et l’augmentation des lacticodeshydrogenases (LDH), un SH a ete suspecte et confirme par le myelogramme. L’evolution a ete favorable sous fortes doses de corticoides. Pres de 80 cas de pancreatites ont ete rapportes au cours du LES. L’etiologie est parfois difficile a etablir : le LES lui meme, les traitements et en particulier les corticoides, dont la responsabilite est controversee, et les infections favorisees par l’immunodepression. Quelques 40 cas de SH sont rapportes au cours du LES parfois en association avec une infection. La mortalite est de 38,7 %. Mais lorsque le SH constitue la manifestation initiale du LES ou survient au cours d’une poussee evolutive, l’evolution peut etre favorable sous corticoides.Exceptionally, acute pancreatitis and reactive hemophagocytic syndrome (RHS) are observed in the course of systemic lupus erythematosus (SLE). However, the association of the two conditions has never been reported before. A 31-years-old woman with a 7-year history of SLE was admitted for abdominal pain and fever. Elevated serum amylase and pancreatic enlargement on computerized tomography confirmed the diagnosis of pancreatitis. Laboratory examinations revealed pancytopenia, abnormal hepatic tests, and elevation of serum LDH and triglyceride levels. Bone marrow aspiration showed hemophagocytosis. The patient responded well to high dose corticosteroids. About eighty cases of pancreatitis have been reported in patients with SLE. The mechanisms are still unclear: SLE as the primary etiologic factor, drug toxicity, especially steroids which play a controversial role, or infection. About 40 cases of RHS have been reported in patients with SLE, sometimes associated with active infection. Overall mortality is 38.5%. When RHS occurs as an initial manifestation of SLE, or in the course of active SLE, it responds well to immunosuppressive therapy.

Le syndrome de Eagle : à propos d'un cas

Le syndrome de Eagle appele egalement elongation du processus styloide est une cause frequente de cervicalgie et de douleur cranio-faciale. C’est une entite assez frequente mais peu connue qui pose un probleme de diagnostic differentiel avec de nombreuses pathologies ORL et maxillo-faciales. Les auteurs rapportent un nouveau cas clinique et illustrent l’apport de la radiologie dans le diagnostic positif, ils presentent en outre une revue de la litterature.

Pachydermopériostose : à propos de deux cas

Pachydermoperiostosis or primary hypertrophic osteoarthropathy is a benign genetically determined disease, manifested by finger clubbing, hypertrophic skin changes and periosteal bone formation. It is rarely reported and was for a long time misdiagnosed and confused with secondary hypertrophic osteoarthropathy. We present the imaging features in two cases and review the literature.

Tuberculose de la paroi thoracique: à propos d'un cas et revue de la littérature

Summary Tuberculosis of the chest wall — including costal tuberculosis, chronic intercostal and subcostal abscesses, sternal and clavicular tuberculosis — is a rare disease. Its occurence is estimated between 0,25 and 5 percent of the osteoarticular tuberculosis. We report the case of an 18-year-old female who presented with a 9-month history of a chronic peristernal abscess. Culture after needle aspiration of the tumor showed Mycobacterium tuberculosis . There were no evidence of any other localisation of tuberculosis. Starting from this case, we recall the pathogenic aspects of the tuberculosis of the chest wall, as well as its clinical features, therapeutics and course.

Profil étiologique des nécroses digitales des membres supérieurs : analyse de 25 observations

AIM To investigate the etiologies of the upper limb digital necrosis based on a retrospective analysis of 25 cases. PATIENTS AND METHODS We retrospectively reviewed the medical records of patients treated for digital necrosis of the upper limb in four departments of internal medicine from January 1997 to December 2003. RESULTS There were 16 women and nine men, mean age 55 years. Eleven patients were smokers. Raynauds phenomenon was noted in 12 cases. Connective tissue diseases were the most common cause (nine cases), all of them were women. The second cause was atherosclerosis (five cases) and Buergers disease (five cases). In the other cases, the following diagnoses were found: vasculitis (three cases) and neoplasm (two cases). No cause could be identified in one female smoker. CONCLUSION Digital necrosis is a common symptom, revealing a vascular pathology. Its causes are diverse. In women, it first suggests a connective tissue disease whereas in men, a diffuse arteriopathy. The etiological diagnosis strategy should consider drug intake, anamnesis and Raynauds phenomenon history. However, in all cases the etiology investigations should not delay the treatment in order to preserve functional prognosis.

Association artérite de Takayasu et leucémie aiguë myéloblastique

Resume Introduction Nous rapportons une observation originale par la survenue d’une leucemie aigue myeloblastique deux mois apres les premieres manifestations vasculaires d’une arterite de Takayasu. Exegese Une patiente de 21 ans, aux antecedents d’erytheme noueux et d’episclerite, a ete hospitalisee pour tumefaction cervicale gauche en rapport avec un anevrisme de la carotide interne gauche. L’exploration vasculaire a en outre mis en evidence une stenose de l’artere sous-claviere gauche. L’examen anatomopathologique de l’anevrisme a montre une arterite granulomateuse a cellules geantes, concluant au diagnostic d’arterite de Takayasu. Deux semaines apres sa sortie, une numeration formule sanguine a montre une hyperleucocytose a 440 000 elements/mm 3 en rapport avec une leucemie aigue myeloblastique. La patiente est decedee d’une hemorragie intracerebrale. Conclusion Des vascularites leucocytoclasiques et des periarterites noueuses ont ete decrites au cours des leucemies aigues myeloblastiques, mais l’association a une arterite de Takayasu est exceptionnelle. A notre connaissance, seules deux observations faisant etat d’une telle association ont ete publiees. (J Mal Vasc 2006; 31: 280-283)

Vascularite urticarienne hypocomplémentémique de McDuffie associée à un syndrome de Gougerot Sjögren

We report a patient with Sjögrens syndrome who presented with urticarial hypocomplementemic vasculitis. A 46-year-old female was admitted for assessment of ascitis. Clinical examination and computed tomographic scan disclosed evidence of multiple peripheral and intra abdominal lymph nodes. During her admission, she developed several bouts of acute angioedema and urticarial skin lesions. Minor salivary gland biopsy showed focal sialadenitis, stage IV of Chisholm. Schirmers test was positive. Laboratory examination found low levels of C1q and high levels of C1q antibodies. Therapy with prednisone and hydroxychloroquine was initiated. Six months later, the patient presented with lower limb oedema. Urinalysis showed proteinuria (1g/day) and renal biopsy revealed membranous nephropathy with favorable outcome with corticosteroids.

Imagerie de la pyélonéphrite xantogranulomateuse

Resume La pyelonephrite xantogranulomateuse pseudotumorale (PXG PT) est une forme particuliere et rare d’infection chronique du rein dont le diagnostic preoperatoire est difficile en raison de l’absence de signe de specificite. Le but de cette etude retrospective de 8 observations de PXG PT est d’evaluer l’apport des differentes methodes d’imagerie dans cette pathologie. Il s’agissait de 6 hommes et de 2 femmes âges de 29 a 75 ans. L’atteinte etait droite dans 5 cas, gauche dans 3 cas, polaire superieure dans 2 cas et polaire inferieure dans 6 cas. L’association a un calcul a ete retrouvee dans 3 cas. L’echographie abdominale a permis de visualiser une formation hypoechogene heterogene (n = 3), hypoechogene homogene (n = 3), isoechogene (n = 1), et paraliquidienne (n = 1). La TDM abdominale a objective une lesion focale tissulaire rehaussee en peripherie (n = 4) et une masse paraliquidienne rehaussee en peripherie (n = 3). L’echographie de la masse tumorale n’a pas de caracteristique propre. La TDM montre la masse renale qui n’a pas de specificite particuliere, le syndrome obstructif et apprecie l’extension peri-renale. L’IRM, non realisee dans notre serie, permet de montrer des signes specifiques du signal de la graisse permettant de suspecter le diagnostic en preoperatoire. C’est donc un faisceau d’arguments cliniques et biologiques associes a la decouverte d’une masse renale focale sans specificite echographique ou tomodensitometrique et contenant un signal de graisse en IRM qui permet de suspecter fortement le diagnostic. La ponction biopsie peut s’averer necessaire dans certains cas pour finaliser le diagnostic et adapter la therapeutique necessaire.