Shulman syndrome. A report of 4 cases and review of the literature

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 fasciitis, suspected on hypereosinophilia, was confirmed by histological findings of muscle biopsy. In the second and the third case, the induration affected arms and legs. Obiouvs streneous exercise was noted in the third patient. Those patients fullfiled the criteria of eosinopfilic fasciitis. 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-like on the extremitis with extension to abdomen. Erythrocyte sedimentation rate was normal. Histological findings confirm the diagnosis of eosinophilic fasciitis. All patients were treated with general steroids at high doses associated to cimetidine in the second patient. Once therapy ended, relapses occur in escond and third cases.