[Shulman syndrome: a review of 4 cases]

The goal of this study is to report four cases of Shulman syndrome with a literature review. Shulman syndrome is a rare disorder recently considered a systemic disease. Our first case shows woody induration of the buttock and trunk with features of morphea. The diagnosis of eosinophilic fascitis, suspected on hypereosinophilia, was confirmed by histological findings of muscle biopsy. In the second and the third case, the induration affected arms and legs. Obvious strenuous exercise was noted in the third patient. Those patients full filed the criteria of eosinopfilic fascitis. Visceral involvement consisted on restrictive lung function defects on the second case and oesophageal hypokinesia in the third case. In the fourth case, there was a scleroderma-Iike on the extremitis with extension to abdomen. Erythrocyte sedimentation rate was normal. Histological findings confirm the diagnosis of eosinophilic fascitis. All patients were treated with general steroids at high doses associated to cimetidine in the second patient. Once therapy ended, relapses occur in second and third cases

Esophageal Involvement in Behcet's Disease

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.

Heparinotherapie et grossesse

The use of anticoagulants in pregnancy problematic. In this focus, the authors reviewed the problems associated with the use of vitamine k antagonist during pregnancy. Then, they presented the general considerations on the use of unfractioned heparin and low molecular weight heparin during pregnancy. Finally they established the therapeutic recommendations for anticoagulation during pregnancy including treatment and prophylaxis of venous thromboembolism, anticoagulation of pregnant women with mechanical heart valves and the management of pregnant women with antiphospholipid antibodies and multiple pregnancy losses