Shulman's syndrome (eosinophilic fasciitis)

摘要

Eosinophilic fasciitis is a rare disease characterized by edema, painful indurations, and progressive muscle weakness. Mainly the extremities are involved. We report on a 22-year-old woman with eosinophilic fasciitis presenting with progressive muscle weakness of both hands and feet and a reduced general condition. She showed symmetrical and firm swelling of the extremities with painful restriction of joint movement. Systemic treatment with glucocorticosteroids as well as physiotherapy and manual lymphatic drainage led to continuous improvement of her symptoms. The differentiation from other diseases, such as systemic scleroderma, eosinophilia-myalgia syndrome, and pseudoscleroderma, might be difficult at the beginning of the disease. The gold standard for diagnosis is--as was done in our case--a deep skin-to-muscle biopsy. Further imaging, especially magnetic resonance imaging, can support the diagnostic procedure.