Eosinophilic fasciitis. Increased collagen production and type I procollagen messenger RNA levels in fibroblasts cultured from involved skin. 1990

V M Kähäri, and J Heino, and L Niskanen, and J Fräki, and J Uitto
Department of Dermatology, Jefferson Medical College, Philadelphia, Pa.

Two patients with eosinophilic fasciitis were studied to elucidate the activation of collagen production in this disorder. Histologic examination of biopsy specimens from the involved area of skin revealed the presence of inflammatory cell infiltrates and various degrees of collagen accumulation in the dermis, subcutis, fascia, and underlying muscle. Fibroblast cultures initiated from the involved skin exhibited 2.0- to 3.7-fold increased collagen production when compared with control fibroblast cultures established from the uninvolved skin of the same patients. Eosinophilic fasciitis fibroblasts also displayed 2.4- to 6.2-fold higher steady-state levels of type I procollagen messenger RNA than did the control cells, indicating pretranslational activation of type I procollagen gene expression. In addition, cellular fibronectin messenger RNA steady-state levels were elevated 1.9- to 3.3-fold in eosinophilic fasciitis fibroblasts. These results suggest that fibroblasts in the involved skin of patients with eosinophilic fasciitis exhibit an activated phenotype, similar to that of scleroderma fibroblasts, leading to accumulation of collagen in the skin and the underlying structures.

UI MeSH Term Description Entries
D008297 Male Males
D002478 Cells, Cultured Cells propagated in vitro in special media conducive to their growth. Cultured cells are used to study developmental, morphologic, metabolic, physiologic, and genetic processes, among others. Cultured Cells,Cell, Cultured,Cultured Cell
D003094 Collagen A polypeptide substance comprising about one third of the total protein in mammalian organisms. It is the main constituent of SKIN; CONNECTIVE TISSUE; and the organic substance of bones (BONE AND BONES) and teeth (TOOTH). Avicon,Avitene,Collagen Felt,Collagen Fleece,Collagenfleece,Collastat,Dermodress,Microfibril Collagen Hemostat,Pangen,Zyderm,alpha-Collagen,Collagen Hemostat, Microfibril,alpha Collagen
D004802 Eosinophilia Abnormal increase of EOSINOPHILS in the blood, tissues or organs. Eosinophilia, Tropical,Hypereosinophilia,Tropical Eosinophilia,Hypereosinophilias,Tropical Eosinophilias
D005208 Fasciitis Inflammation of the fascia. There are three major types: 1, Eosinophilic fasciitis, an inflammatory reaction with eosinophilia, producing hard thickened skin with an orange-peel configuration suggestive of scleroderma and considered by some a variant of scleroderma; 2, Necrotizing fasciitis (FASCIITIS, NECROTIZING), a serious fulminating infection (usually by a beta hemolytic streptococcus) causing extensive necrosis of superficial fascia; 3, Nodular/Pseudosarcomatous /Proliferative fasciitis, characterized by a rapid growth of fibroblasts with mononuclear inflammatory cells and proliferating capillaries in soft tissue, often the forearm; it is not malignant but is sometimes mistaken for fibrosarcoma. Fascitis,Fasciitides,Fascitides
D005260 Female Females
D005347 Fibroblasts Connective tissue cells which secrete an extracellular matrix rich in collagen and other macromolecules. Fibroblast
D005353 Fibronectins Glycoproteins found on the surfaces of cells, particularly in fibrillar structures. The proteins are lost or reduced when these cells undergo viral or chemical transformation. They are highly susceptible to proteolysis and are substrates for activated blood coagulation factor VIII. The forms present in plasma are called cold-insoluble globulins. Cold-Insoluble Globulins,LETS Proteins,Fibronectin,Opsonic Glycoprotein,Opsonic alpha(2)SB Glycoprotein,alpha 2-Surface Binding Glycoprotein,Cold Insoluble Globulins,Globulins, Cold-Insoluble,Glycoprotein, Opsonic,Proteins, LETS,alpha 2 Surface Binding Glycoprotein
D005786 Gene Expression Regulation Any of the processes by which nuclear, cytoplasmic, or intercellular factors influence the differential control (induction or repression) of gene action at the level of transcription or translation. Gene Action Regulation,Regulation of Gene Expression,Expression Regulation, Gene,Regulation, Gene Action,Regulation, Gene Expression
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man

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