Eosinophilic Fasciitis Treatment & Management

Updated: Jan 03, 2017
  • Author: Peter M Henning, DO; Chief Editor: Herbert S Diamond, MD  more...
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Treatment

Approach Considerations

First-line therapy for eosinophilic fasciitis is with systemic corticosteroids. [5] Although patients may require prolonged therapy, it should be noted that up to one third of eosinophilic fasciitis cases may spontaneously resolve. [54]

Case reports describe the use of a number of agents for second-line therapy. No consensus exists on which agent is best for that purpose.

Physical therapy should be initiated to improve joint mobility and to decrease contractures.Surgical release has been used in some cases to manage significant joint contractures. [55]

Dermatologists, rheumatologists, and surgeons (for the skin-muscle biopsy) are consulted most often for management of these cases.

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Medical Care

Initial therapy

There is wide consensus that systemic corticosteroids are the initial therapeutic agent of choice. Typically, prednisone or prednisolone is used, in doses ranging from 20-100 mg/day. Response is considered satisfactory with reduction in edema, improvement in skin thickening, resolution of carpal tunnel syndrome, and gradual decrease in joint contracture. Eosinophilia and inflammatory markers frequently resolve promptly after initiation of prednisone therapy. [18, 19, 20, 39, 50, 56, 5]

Additional therapeutic agents and adjunctive therapies

Multiple additional therapeutic agents have been used as disease-modifying or steroid-sparing agents in persistent or steroid-resistant cases of eosinophilic fasciitis. It should be noted that there is no general consensus with regard to the best agent for this type of disease. Treatment numbers are generally small, and controlled trials are lacking. [19, 57, 58, 59, 60]

Case reports detail the use of multiple additional agents, including antihistamines, cimetidine, hydroxychloroquine, chloroquine, azathioprine, cyclosporine, dapsone, infliximab, tacrolimus, methotrexate, D-penicillamine, griseofulvin, ketotifen, and alpha-interferon, with varying rates of response. Some data suggest that other anti–tumor necrosis factor (TNF)–alpha agents may also be beneficial. [54]

One study reviewed the treatment modalities used in 32 adult patients with biopsy-proven eosinophilic fasciitis. All patients received corticosteroids as a first-line therapy. Fifteen patients (47%) received methylprednisolone pulses at treatment initiation, and 14 patients (44%) received an immunosuppressive agent, usually methotrexate (86%), as a second-line therapy. There was complete remission in 69% of patients; remission with disability in 19%; and failure in 12%. A poor outcome was associated with a delay in diagnosis greater than 6 months and lack of methylprednisolone pulses. [61]

In a review of 63 patients with eosinophilic fasciitis, Wright and colleagues reported a higher rate of complete response in patients treated with the combination of corticosteroids and methotrexate (21 of 33 patients), compared with other treatment combinations, corticosteroids only, or treatment without corticosteroids. [5]

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