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Eosinophilic fasciitis in children frequently progresses to scleroderma-like cutaneous fibrosis
M L Farrington1, J E Haas, V Nazar-Stewart
1Department of Pediatrics, University of Washington, Seattle 98195.
Insights
Pediatric eosinophilic fasciitis (EF) can lead to lasting skin fibrosis, especially in younger children. Early, limited disease presentation is linked to complete resolution in children with EF.
Area of Science:
- Pediatric Rheumatology
- Dermatology
- Immunology
Background:
- Eosinophilic fasciitis (EF) is a rare inflammatory condition affecting the fascia.
- Long-term outcomes in pediatric patients with EF are not well-established.
- Understanding factors influencing disease progression is crucial for patient management.
Purpose of the Study:
- To evaluate the long-term outcomes of pediatric eosinophilic fasciitis.
- To identify predictors of disease progression to cutaneous fibrosis.
- To analyze the relationship between initial disease extent and final outcome.
Main Methods:
- Retrospective case review of 21 pediatric patients with biopsy-proven EF.
- Analysis of data from 4 patients followed in a rheumatology clinic and 17 from literature.
- Comparison of outcomes based on age at diagnosis, initial disease severity, and other clinical factors.
Main Results:
- Two-thirds of pediatric EF patients developed residual cutaneous fibrosis.
- Children under 7 years were twice as likely to develop fibrosis (RR=2.0).
- Patients with extensive disease at diagnosis (3-4 extremities +/- trunk) invariably progressed to fibrosis, while those with minimal disease (1-2 extremities) resolved completely.
Conclusions:
- Age and initial disease extent are significant predictors of long-term outcome in pediatric EF.
- Younger children and those with extensive disease at onset are at higher risk for developing cutaneous fibrosis.
- Early recognition and management of extensive pediatric EF may be critical to prevent long-term sequelae.
Abstract:
We evaluated longterm outcome in 21 pediatric patients with biopsy proven eosinophilic fasciitis (EF), 4 followed in our rheumatology clinic and 17 reported in the literature. Two-thirds of these patients developed residual cutaneous fibrosis, while one-third enjoyed complete resolution of disease. Children under age 7 years were twice as likely as those over age 7 years to experience disease progression to cutaneous fibrosis [relative risk = 2.0 (95% confidence intervals 1.2, 3.4)]. Fourteen of 17 patients with extensive disease at diagnosis (involvement of 3-4 extremities +/- trunk) progressed to cutaneous fibrosis whereas all 4 patients with minimal disease (involvement of 1-2 extremities) at onset resolved completely. We detected no association between progression to cutaneous fibrosis and sex of patient, duration of symptoms prior to therapy, type of therapy, history of prior physical stress, or laboratory variables at diagnosis.