Pathology of eosinophilic fasciitis and its relation to polymyositis.
ABSTRACT The anatomical substrate of eosinophilic fasciitis (EF) was studied in 15 muscle biopsy specimens of this disease, six of which included the dermis and subcutaneous tissue. As controls, 94 postmortem muscle specimens from patients dying of non-muscular diseases were used. Of these 94 specimens, 22 (23.4%) showed practically no deep fascia and 72 specimens showed a single dense bundle of collagen with no distinction between deep fascia and epimysium. The 15 specimens of EF showed thickening and inflammatory infiltration of varying degrees in the deep fascia, epimysium, perimysium, endomysium and also in muscle. We conclude that the anatomical substrate of EF is not confined to the deep fascia, but involves other structures including mysia and muscle itself. Most reported cases of EF in the literature do not even describe muscle. A comparative study of 15 biopsy specimens of polymyositis and dermatomyositis with those of EF revealed only quantitative differences in the histopathological changes of muscle and mysia, inflammatory infiltrate and eosinophilia. We suggest that the diseases are more closely related than previously recognized.
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ABSTRACT: The eosinophilia-myalgia syndrome (EMS) is a newly recognized illness that occurred in an epidemic form during the summer of 1989. The illness was characterized in the acute phase by myalgia and eosinophilia, followed in many patients by chronic cutaneous lesions, progressive neuropathy, and myopathy. EMS was associated with ingestion of L-tryptophan, an essential amino acid marketed as a nutritional supplement but widely used as a therapeutic agent. Evidence of abnormal L-tryptophan metabolism has been described in patients with EMS, and most likely reflects increased activity of indoleamine 2,3-dioxygenase, the rate-limiting enzyme of tryptophan metabolism. A contaminant identified in EMS-associated L-tryptophan preparations has been isolated and characterized, but its biologic effects and role as the etiologic agent in EMS remain to be established. Pathologic observations and experimental studies indicate that eosinophils, mononuclear inflammatory cells, and fibroblasts are potential effector cells, and interleukin-5 and transforming growth factor-beta are important mediators in the pathogenesis of the syndrome. Although few new cases of EMS occurred following the withdrawal of L-tryptophan, affected patients continue to manifest late sequelae of the disease, including dermal fibrotic conditions. This tragic outbreak of a newly recognized illness has focused interest on the role of chemical and environmental agents in the pathogenesis of various idiopathic illness characterized by tissue inflammation and fibrosis.Journal of Investigative Dermatology 02/1993; 100(1):97S-105S. · 6.19 Impact Factor
- The Journal of Dermatology 07/2009; 36(6):358-9. · 1.77 Impact Factor
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ABSTRACT: Inflammatory fibrotic disorders have been of high interest both for dermatologists and rheumatologists. Although the phenotypic end stage of this group of diseases is ultimately the same, namely fibrosis, patients present with different clinical features and are often treated with distinct therapeutic modalities. This review addresses whether there is evidence for different underlying molecular pathways in the various inflammatory fibrotic diseases such as localized scleroderma, pediatric lichen sclerosus, adult lichen sclerosus, eosinophilic fasciitis and systemic sclerosis. To investigate this, a large number of gene expression microarray studies performed on skin or fibroblasts from patients with these aforementioned diseases were described, (re-)analysed, and compared. As suspected by the heterogeneous phenotype, most diseases showed unique gene expression features. Intriguingly, a clear overlap was observed between adult and pediatric lichen sclerosus and localized scleroderma, in antigen processing and the interferon pathway. Delineating the cause and consequence of these pathways may generate novel tools to better characterize and more effectively treat these patients.Expert Review of Clinical Immunology 01/2014; · 2.89 Impact Factor