Eosinophilic Fasciitis: Current concepts of skin and systemic manifestations

Publication date: Available online 10 April 2018
Source:Clinics in Dermatology
Author(s): Nicole Fett, Megan Arthur
Eosinophilic fasciitis (EF) is an uncommon connective tissue disease characterized by abrupt onset of edema, followed by progressive induration of primarily the distal extremities. Patients may exhibit inflammatory arthritis, joint contractures, decreased mobility, and nerve entrapment. Almost half of patients with EF may have coexisting morphea plaques. Classic laboratory studies display peripheral eosinophilia, hypergammaglobulinemia, and elevated inflammatory markers. EF is included in the spectrum of scleroderma-like disorders and may be difficult to distinguish from other sclerosing skin disorders. Full thickness biopsy containing muscle and fascia is considered the gold standard for diagnosis and reveals sclerosis of the mid-deep dermis, subcutaneous fat, and thickening of the fascia. MRI has been increasingly utilized to augment diagnostic capabilities. Ultimately, the diagnosis of EF relies upon the combination of characteristic clinical, laboratory, imaging, and histologic findings. While some patients experience spontaneous remission, systemic corticosteroids (SCS) are the mainstay of treatment. Patients who fail to improve with SCS alone require the addition of a second immunosuppressive drug. Additionally, although data are limited, there is evidence to suggest that initial combination therapy with SCS, and methotrexate may be most beneficial.