Eosinophilic Fasciitis Follow-up

Updated: Dec 06, 2018
  • Author: Peter M Henning, DO; Chief Editor: Herbert S Diamond, MD  more...
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Follow-up

Further Outpatient Care

Patients with eosinophilic fasciitis (EF) should continue to be treated with corticosteroid therapy in the outpatient setting.

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Prognosis

A retrospective review found that clinical factors associated with persistent fibrosis included presence of morphealike skin lesions, younger age at onset, truncal involvement, and presence of dermal fibrosclerosis on histopathologic specimen. [5]

Loss of edema is usually the first clinical sign of improvement and can occur within 4 weeks of commencing treatment. Concurrently, the skin becomes softer, but 3-6 months may elapse before maximal reduction in induration and contractures is achieved. [19, 21]

While total resolution of the clinical signs can occur, some degree of induration remaining even after many months of corticosteroid therapy is not unusual.

A direct correlation does not always exist between clinical disease activity and laboratory findings. The eosinophilia and ESR usually return to reference ranges within 6-8 weeks, although the ESR and hypergammaglobulinemia may remain abnormal for up to 12 months. [19, 21]

Eventually, corticosteroid therapy can be withdrawn in many of the patients, without relapse occurring.

The development of aplastic anemia is a rare but grave complication. [44] One study reported on 4 patients with eosinophilic fasciitis and severe aplastic anemia. In 3 cases, the aplastic anemia was refractory to conventional immunosuppressive therapy with antithymocyte globulin and cyclosporine. However, in 1 patient, rituximab displayed significant efficacy for both the skin and hematologic symptoms. In an additional 19 cases of eosinophilic fasciitis and aplastic anemia, corticosteroid regimens improved skin symptoms in 5 of 12 cases but were ineffective in the treatment of aplastic anemia in all but 1 case. Aplastic anemia was profound in 13 cases and was the cause of death in 8 cases. Only 5 patients achieved long-term remission. [65]

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