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.
- The Journal of Dermatology 07/2009; 36(6):358-9. · 2.35 Impact Factor
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ABSTRACT: Study of 18 biopsy specimens in 11 patients with L-tryptophan-related eosinophiliamyalgia (fasciitis) syndrome showed hyaline sclerodermoid changes. Dermal scleroderma was found in eight of nine punch biopsy specimens and eight of nine excisional biopsy specimens. Fascial scleroderma was found in eight excisional biopsy specimens. One specimen obtained by excision had no fascia present. Eleven biopsy specimens showed edema of the dermis, and 13 showed dilated lymphatic structures; thus, the clinical picture of edematous sclerosis was confirmed. Mucinous fasciitis was present in five excisional biopsy specimens, in conjunction with a large number of macrophages in four. Dermal mucinosis was present in 11 biopsy specimens. Lymphocytic and macrophage inflammation was minimal in 14 biopsy specimens and pronounced in only 4. Plasma cells were present in eight cases. Eosinophils were present in substantial numbers in three biopsy specimens and only occasionally in four. Eosinophilic spongiosis was observed in one patient. Lymphocytic inflammation was noted around a single muscle spindle and around large nerve trunks in three patients. No relationship was established between these pathologic features and the duration or dose of tryptophan, prednisone treatment, or duration of symptoms. Pathologic features of the L-tryptophan syndrome consist of hyaline sclerodermoid collagen in the dermis, the septa, and the fascia. Edema, focal mucinosis, and macrophage inflammation may be features that identify this event.Mayo Clinic Proceedings 06/1991; 66(5):457-63. · 5.79 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