Dermatologic Manifestations of Eosinophilic Fasciitis Treatment & Management

Updated: Jan 31, 2017
  • Author: Brad S Graham, MD; Chief Editor: William D James, MD  more...
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Treatment

Medical Care

Many eosinophilic fasciitis cases respond to corticosteroids (88%, with 25% obtaining complete recovery), although spontaneous resolution is possible. Complete recovery may take up to 1-3 years. No consensus on the treatment of eosinophilic fasciitis exists, but most studies indicate that the best response is with moderate-to-high doses of corticosteroids, especially if started early in the disease course. No set dosing schedule is available, but most studies advocate doses of 0.5-1 mg/kg/d until response, with rapid tapering to alternate day therapy.

Several eosinophilic fasciitis cases exist in the literature of recalcitrant disease to corticosteroids in which adjunctive therapy may be required.

Adjunctive medications for eosinophilic fasciitis include hydroxychloroquine, colchicine, cimetidine, cyclosporin, [24] azathioprine, and methotrexate. A more recent study looked at extracorporal photochemotherapy in the treatment of corticosteroid-resistant cases. After 1 year of therapy, 2 of 3 patients showed considerable improvement when combined with low-dose corticosteroids. [25]

Newer therapies that have been used as corticosteroid adjuncts or as monotherapy for eosinophilic fasciitis include infliximab, [26] cyclophosphamide, [27] dapsone, [28] retinoid-UVA1, [29] and oral psoralen plus UVA (PUVA). [30]

In a promising follow-up report on three cases of steroid-resistant eosinophilic fasciitis, all patients showed improvement in skin induration and joint contractures with infliximab at 3 mg/kg every 8 weeks. All patients were in remission in 1-3 years. [31]

A case report in 2016 ironically noted the onset of eosinophilic fasciitis during infliximab therapy for psoriatic arthritis. [32]

A single case report in 2009 reported the use of intravenous immunoglobulin to treat eosinophilic fasciitis. [33]

In a large series of 34 patients with eosinophilic fasciitis, corticosteroids was the standard first-line therapy. In this series, 44% of patients received a second-line therapy adjunctive immunosuppressant, usually methotrexate. The highest chance of complete remission was in patients treated with pulse methylprednisolone at the earliest onset of diagnosis. This also minimized the need for additional immunosuppressive drugs. [34]

A case report in 2102 documented 2 patients with corticosteroid-refractory eosinophilic fasciitis who were successfully treated with D-penicillamine. [35]

A case report in 2015 described a relapse post corticosteroid treatment, which became steroid-resistant and refractory to methotrexate and antitumor necrosis factor agent treatment. This patient had a rapid response with sustained remission to the anti-interleukin 6 agent, tocilizumab. [36]

A study in 2016 of 12 patients with eosinophilic fasciitis refractory to corticosteroids and weekly methotrexate were treated with high-dose intravenous pulse methotrexate. Methotrexate was dosed at 4 mg/kg monthly (followed by folinic rescue) for 5 months. Skin scores and range of motion improved significantly. [37]

A 2016 case report discussed successful treatment of a patient with refractory eosinophilic fasciitis with sirolimus at 2 mg/d. After 9 months, the patient had a significant reduction in skin thickening. Therapy also allowed for a reduction of the prednisone dose. [38]

 

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Surgical Care

Surgical decompression of carpal tunnel syndrome may be required for eosinophilic fasciitis.

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Consultations

A physical therapist may be consulted. Active and passive range of motion therapy of the involved extremities and joints is crucial along with medical therapy to prevent and to treat joint contractures.

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