In the Thick of It: A Case Report of Eosinophilic Fasciitis, a Great Mimicker of Systemic Sclerosis

Summary: Eosinophilic fasciitis (EF), also known as Shulman’s disease, is a rare autoimmune disorder that is characterized by inflammation and thickening of the skin and underlying connective tissues. The hallmark feature of EF is the infiltration of eosinophils into the affected tissues. The exact etiology and pathogenesis are not well understood but is believed to involve an abnormal immune response causing inflammation and fibrosis. Some researchers suggest that triggers could include environmental factors or infections, especially in genetically predisposed individuals, although most cases are idiopathic. Diagnosis is based on a combination of clinical symptoms, physical exam findings of skin induration with an “orange peel” appearance, lab results showing eosinophilia with increased inflammatory markers, including MRI, and biopsy of affecting tissue including skin and muscle biopsy. We present the case of a 60 year-old woman whose diagnosis eluded clinicians for a long period due to overlapping clinical features of EF and systemic sclerosis.