PURPOSE: Eosinophilic fasciitis or Shulman's disease is a rare condition of unknown etiology. METHODS: We report a retrospective case series of 11 patients with eosinophilic fasciitis (seven men and four women, including a single pediatric case) and perform a systematic literature review to determine the main features of this disease. RESULTS: Mean age of the patients was 46 years. Subcutaneous induration of limbs observed in all the patients was the major presenting symptom. The induration was atypically located in the chest area in two patients. Blood eosinophilia was absent in five cases. Histological fasciitis was demonstrated in all patients and eosinophilic infiltration was present in seven patients. Relapse of subcutaneous induration was observed in only one patient who gradually developed systemic sclerosis. CONCLUSION: Diagnosis of eosinophilic fasciitis should be considered in the presence myalgia and subcutaneous induration of limbs, blood eosinophilia and hypergammaglobulinemia. Treatment is based on systemic corticosteroids. Copyright (c) 2010. Published by Elsevier SAS.
PURPOSE:Eosinophilic fasciitis or Shulman's disease is a rare condition of unknown etiology. METHODS: We report a retrospective case series of 11 patients with eosinophilic fasciitis (seven men and four women, including a single pediatric case) and perform a systematic literature review to determine the main features of this disease. RESULTS: Mean age of the patients was 46 years. Subcutaneous induration of limbs observed in all the patients was the major presenting symptom. The induration was atypically located in the chest area in two patients. Blood eosinophilia was absent in five cases. Histological fasciitis was demonstrated in all patients and eosinophilic infiltration was present in seven patients. Relapse of subcutaneous induration was observed in only one patient who gradually developed systemic sclerosis. CONCLUSION: Diagnosis of eosinophilic fasciitis should be considered in the presence myalgia and subcutaneous induration of limbs, blood eosinophilia and hypergammaglobulinemia. Treatment is based on systemic corticosteroids. Copyright (c) 2010. Published by Elsevier SAS.