[Polymyositis and dermatomyositis in adults. Tunisian multicentre study]. / Les polymyosites et dermatomyosites de l'adulte. Etude multicentrique tunisienne.

INTRODUCTION: To study the clinical features and the outcome of polymyositis (PM) and dermatomyositis (DM) in Tunisia. METHODS: Retrospective multicentre study conducted by the Tunisian Society of Internal Medicine. Adult patients with confirmed and probable PM and DM, according to the Bohan and Peter's criteria, were included in the study and followed-up between 1987 and 2005. RESULTS: Twenty cases of PM and 50 cases of DM were included. Female/male sex-ratio was 2.5. The mean age at diagnosis was 40.7 years. Muscle weakness concerned girdle muscles in 94.3% and pharyngeal muscles in 42.8% of the patients. Interstitial lung disease and heart involvement occurred respectively in 35.7 and 20%. Eyelid erythema, peri-ungual erythema and Gottron's sign occurred in respectively 86, 50 and 36% of the cases of DM. Malignant disease was found in 12.8% of the patients (mainly gynecological and nasopharyngeal cancers). All patients received high dose corticosteroids with additional immunosuppressive drugs in 40% of the cases. The outcome was stabilization or partial improvement in 71.5% of the cases. Death occurred in 20% and was caused by respiratory failure, sepsis and cardiac failure. CONCLUSION: The particularities of PM and DM in Tunisia are the preponderance of DM, early onset of the disease and high frequency of the nasopharyngeal cancer.

[Primary chronic autoimmune neutropenia successfully treated with sirolimus]. / Neutropénie auto-immune primaire avec autoanticorps antineutrophiles traitée avec succès par sirolimus.

We report a 74-year-old man with severe chronic primary neutropenia (neutrophil count: 390 per millimeter cube) uncovered following surgery for perianal abscess collection. Clinical, laboratory and roentgenographic findings revealed no abnormality. Antineutrophil antibodies were positive in two consecutive serum samples. Under cyclosporine, neutrophil count reached 1970 per millimeter cube. However, this therapy was discontinued due to new onset of severe renal failure. After six weeks, neutrophil count was 950 per millimeter cube and sirolimus was started, resulting in renal function improvement and resolution of neutropenia.

[Hereditary multiple exostosis revealed by deep vein and arterial popliteal thrombosis]. / Maladie des exostoses multiples révélée par une thrombose poplitée veineuse et artérielle.

We report a 38-year-old man who presented successively a thrombosis of the right popliteal vein and the right popliteal artery, responsible of a painful edema of the leg. There was no known thrombophilic predisposing condition. Radiologic assessment, guided by clinical findings, evidenced bilateral femoral and tibial exostosis resulting in a diagnosis of hereditary multiples exostosis. Although anticoagulant treatment with unfractioned heparin was instituted, distal ischemia of the right lower limb developed. Resection of the compressive osteochondroma and an arterial femorotibial bypass venous graft were performed in addition to prolonged anticoagulant therapy. Outcome was eventually favorable. Hereditary multiple exostosis may be asymptomatic for long time. Compression of adjacent vascular structures by an osteochondroma is a rare and potentially serious complication.

[Leprosy: a rare imported disease]. / Une maladie d'importation rare: la lèpre.

Although rare in occidental countries, leprosy is an endemic disease throughout the world. Physicians may encounter imported cases and thus need to be aware of this diagnosis. We here report a 41-year-old male patient from French West Indies who presented with nonspecific extensive skin lesions and a peripheral neuropathy. Skin biopsy examination led to the diagnosis of borderline lepromatous leprosy.