The eosinophilia-myalgia syndrome and eosinophilic fasciitis.
The scientific excitement that follows the recognition of a new disease has been reflected in the numerous publications describing the clinical, histopathologic, and pathogenetic aspects of the eosinophilia-myalgia syndrome (EMS) during the period covered by this review. The clinical picture that has emerged during the past 2 years indicates that EMS is a multisystemic disease with prominent cutaneous, hematologic, and visceral manifestations that frequently evolves into a chronic course and can occasionally be fatal. Considerable progress has been made toward understanding the etiology and pathogenesis of EMS. The demonstration of an association with the ingestion of L-tryptophan-containing products originating from a single source has led to the identification and characterization of a putative etiologic agent present as a contaminant in these preparations. Although the accumulation of eosinophils, lymphocytes, macrophages, and fibroblasts in the affected tissues suggests that these cells play important roles in the pathogenesis of EMS, the precise mechanisms of their involvement have not been established. Several studies have demonstrated the activation of eosinophils and the deposition of eosinophil-derived toxic proteins in affected tissues. Fibroblast activation and increased expression of genes coding for various connective tissue macromolecules have been demonstrated employing in situ hybridizations with complementary DNAs. Furthermore, interleukin-5 and transforming growth factor-beta have been implicated as potential mediators in the pathogenesis of EMS. The explosive epidemic of EMS has emphasized the importance of chemical and environmental factors in the development of systemic disorders characterized by chronic inflammation and fibrosis. It is expected that further study of the pathogenesis of EMS will provide valuable information regarding the mechanisms responsible for these obscure disorders.
Jimenez
S A
SA
Thomas Jefferson University, Philadelphia, Pennsylvania.
Varga
J
J
D004802:Eosinophilia / Q000453:epidemiology / Q000209:etiology / Q000473:pathology*; D016603:Eosinophilia-Myalgia Syndrome / Q000453:epidemiology / Q000209:etiology / Q000473:pathology*; D005208:Fasciitis / Q000453:epidemiology / Q000209:etiology / Q000473:pathology*; D006801:Humans