Sarcoidosis with heart involvement: a rare association of terrible prognosis, a report of two cases

Sarcoidosis is a multisystemic disorder of unknown etiology which is characterized by the formation of  non-caseating granulomas in involved tissues. Cardiac involvement is one of the least common manifestations and it can occur at any point of time during the course of sarcoidosis. Here we present the case of 2 patients with known sarcoidosis who develop cardiac abnormalities in the absence of known primary cardiac cause. In our report, we would like to draw attention to the importance of considering heart involvement in any case with systemic sarcoidosis especially in young age.Key words: Cardiac sarcoidosis, Sarcoidosis, Ventricular arrhythmia

Un anévrysme sacciforme de l’aorte abdominale révélant une maladie de Behçet: quand faut-il opérer?

L'atteinte vasculaire est fréquente au cours de la maladie de Behçet. Elle est essentiellement représentée par les thromboses veineuses alors que l'atteinte artérielle est plus rare. Elle peut être isolée ou multifocale et peut toucher tous les territoires avec prédilection pour l’aorte abdominale, les artères pulmonaires et les artères des membres inférieurs. L'atteinte anévrysmale de l'aorte abdominale est trompeuse se manifestant par une symptomatologie atypique responsable d'un retard diagnostique favorisant la rupture. Dans notre cas, des douleurs abdominales paroxystiques ont incité le patient à consulter rapidement. L’enquête étiologique a conclue à un angio Behcet. Rarement, l’anévrysme de l’aorte abdominale est révélé par une complication telle que la rupture dans le rétro péritoine ou le tube digestif. Les indications chirurgicales des anévrysmes de l’aorte sont discutées ici. Un traitement immunosuppresseur au long cours s’impose en post opératoire pour limiter le risque de récidive

WERNER SYNDROME: A NEW CASE REPORT

“Werner’s syndrome” or premature aging syndrome is a rare autosomal recessive genetic disease. It is responsible of several complications related to age, including atherosclerosis and association with cancer. We report the case of a 36 year-old-patient, admitted to department of Internal Medicine of the military hospital of Tunis for suspicion of systemic sclerosis. The patient had all the major signs of Werner syndrome (bilateral cataract, sclerotic skin, “bird face”, baldness, small size, parental consanguinity) and 4 minor signs (type 2 diabetes, hypogonadism, squeaky voice, and flat feet). She has also a brother with the same morphotype died at the age of 32 by a myocardial infarction. The current follow-up time is 9 years.

Symptomatic macroglossia and tongue myositis in Dermatomyositis

The involvement of the tongue in dermatomyositis is rarely described in the literature. We report the case of a patient having a macroglossia whose etiologic was a dermatomyositis. The diagnosis was established by biopsy of the tongue which showed an interstitial lymphocytic infiltration associated with destruction of muscle fibers and perifascicular atrophy. The treatment was based on corticosteroids. The functional prognosis was dominated by the gene to speech and the swallowing disorders