[Myalgia in familial Mediterranean fever]. / Myalgies au cours d'une fièvre méditerranéenne familiale.

INTRODUCTION: Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by recurrent attacks of fever and painful episodes of sterile peritonitis, pleuritis and arthritis. Among rare symptoms of the disease, muscular manifestations, first described in 1945, sometimes as one of the main clinical manifestations or as its sole feature should be recognized. We present a patient with FMF in whom severe myalgia were predominant. CASE REPORT: An 18 year-old Tunisian boy treated with corticosteroids for an "inflammatory myopathy" in another institution was admitted for abdominal pain. FMF was suspected because of a history of paroxysmal abdominal pain with fever from the age of 5 leading two times to laparotomy and one attack of left knee arthritis at the age of 14. FMF diagnosis was confirmed genetically, corticosteroids were tapered and a treatment with colchicine was started. Two years and a half later, he was admitted for severe and incapacitating myalgia of the upper and lower limbs without fever nor abdominal pain that responded well to rest and colchicine. Myalgia was then definitively attached to FMF. CONCLUSION: Three clinical patterns of myalgia are now well identified in FMF: the spontaneous pattern as observed in our patient, the exercise-induced pattern and the protracted febrile myalgia syndrome. The three patterns differ in the severity of pain, grade of fever and duration of the episode.

[A case of papillitis revealing primary syphilis]. / Papillite révélant une syphilis primaire.

OBJECTIVE: Syphilitic ocular manifestations are polymorphous and usually occur during the secondary or tertiary stage of syphilis. We report a case of primary syphilis revealed by papillitis. DESIGN: A 22 year old man presented with blurred vision in the left eye and decreased visual acuity. Fundus examination and fluorescein angiography revealed a papilledema in the left eye and chorioretinitis in the right one. Clinical examination revealed a painless ulceration of the chin. Blood tests were positive for syphilis (positive reaction to the VDRL test and TPHA titer at 1/640) but negative for HIV. After penicillin therapy, the ocular manifestations resolved. RESULTS: Papillitis is a relatively rare ocular manifestation of syphilis. Our case is original because papillitis was the presenting manifestation of the disease and that it was concomitant with the primary chancre. CONCLUSION: Systematic screening for syphilis should be performed in unexplained ocular inflammation.

[Behçet's disease in Tunisia. Clinical study of 519 cases]. / La maladie de Behçet en Tunisie. Etude clinique de 519 cas.

PURPOSE: To describe clinical characteristics of Behçet's disease in Tunisia. METHODS: It's 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.

[Takayasu's arteritis associated with acute myeloblastic leukemia]. / Association artérite de Takayasu et leucémie aiguë myéloblastique.

INTRODUCTION: We present one patient with acute myeloblastic leukemia diagnosed two months after the onset of Takayasu's arteritis. EXEGESIS: A 21-year old woman with a previous history of erythema nodosum and episcleritis was admitted for a left cervical mass. Diagnostic imaging showed an aneurism of the left extracranial internal carotid and a stenosis of the left subclavian artery. Histological findings of the carotid aneurism revealed a granulomatous giant cell arteritis consistent with Takayasu's arteritis. Two weeks after, she was discharged, elevated white cell count (440.000/mm3 ) was disclosed. A bone marrow aspirate documented an acute myeloid leukemia. The patient died of intracerebral hemorrhage. CONCLUSION: Leucocytoclastic vasculitis and polyarteritis nodosa occur in acute myeloid leukemia, but the association with Takayasu's arteritis is new. In our knowledge, only two documented cases of Takayasu's arteritis in association with acute myeloblastic leukemia have been published.

[Intestinal lymphoma associated with Behçet disease]. / Lymphome intestinal associé à une maladie de Behçet.

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.