Eosinophilic Fasciitis in Pediatric Patients: A Rare but Distinct Autoimmune Fibrosing Disorder.

Abstract

Eosinophilic fasciitis (EF), or Shulman syndrome, first described in 1974, is a rare fibrosing disorder characterized by painful, symmetric swelling and progressive woody induration of skin and subcutaneous tissues. Although the pathogenesis remains unclear, EF is considered immune-mediated, often triggered by physical exertion, infections, or medications. Pediatric EF, a particularly uncommon subset, can exhibit distinct clinical features, including pronounced extracutaneous manifestations, unpredictable disease progression, and variable therapeutic responses. Clinical presentations range from rapidly advancing fibrosis leading to joint contractures to fluctuating inflammatory episodes. Diagnosis is challenging because of the absence of universal criteria, although peripheral eosinophilia, elevated inflammatory markers, and imaging findings support clinical suspicion. Definitive diagnosis depends on deep skin and fascial biopsy, revealing eosinophil-rich lymphoplasmacytic infiltrates and fibrosis. Management primarily relies on systemic corticosteroids, supplemented by steroid-sparing immunosuppressive medications in refractory cases. Early diagnosis and treatment are critical because untreated EF can cause irreversible fibrosis and significant functional impairment. The disease's rarity, heterogeneous presentations, and unclear etiology further complicate clinical management. Recent insights suggest EF may involve intricate interactions among environmental triggers, immune dysregulation, and fibrotic remodeling. This review aims to provide an updated overview of pediatric EF, highlighting current knowledge on clinical manifestations, diagnosis, differential diagnosis, therapeutic approaches, and outcomes, supported by an illustrative case, with emphasis on areas needing further research.