transforming-growth-factor-beta and Fasciitis

Systemic plasma cell dyscrasias have diverse manifestations in the skin and include an inflammatory paraneoplastic process. We encountered cases of scleroderma and eosinophilic fasciitis in the setting of an underlying plasma cell dyscrasia.. Ten cases of scleroderma-like tissue reactions in the setting of an underlying plasma cell dyscrasia were encountered. The biopsies were stained for Transforming growth factor (Transforming growth factor) beta, IgG4, kappa, and lambda.. Patients presented with a sclerodermoid reaction represented by eosinophilic fasciitis (5 cases), morphea (3 cases), and systemic scleroderma (2 cases). The mean age of presentation was 70 years with a striking female predominance (4:1). Acral accentuation was noted in 8 cases. In 6 of the cases, the cutaneous sclerosis antedated (4 cases) by weeks to 2 years or occurred concurrently (2 cases) with the initial diagnosis of the plasma cell. The biopsies showed changes typical of eosinophilic fasciitis and/or scleroderma. In 5 cases, light chain-restricted plasma cells were present on the biopsy. There was staining of the plasma cells for Transforming growth factor beta in 3 out of 5 cases tested.. In any older patient presenting with a sudden onset of eosinophilic fasciitis or scleroderma especially with acral accentuation, investigations should be conducted in regards to an underlying plasma cell dyscrasia.

Topics: Aged; Aged, 80 and over; Biomarkers, Tumor; Biopsy; Eosinophilia; Fasciitis; Female; Humans; Immunoglobulin G; Immunoglobulin kappa-Chains; Immunoglobulin lambda-Chains; Immunohistochemistry; Male; Middle Aged; Paraneoplastic Syndromes; Paraproteinemias; Prognosis; Scleroderma, Localized; Scleroderma, Systemic; Skin; Transforming Growth Factor beta

Eosinophilic fasciitis (EF) and peripheral eosinophilia are rare in children.. To describe a case of EF in a 3-year-old child who presented with acute painless induration of her forearm.. Cytokine profiles were obtained and compared with patients with atopic disease during the acute presentation and after treatment with low-dose prednisone.. The patient's serum showed elevation of transforming growth factor beta1 and interleukin-5, which improved after treatment with low-dose prednisone.. The case suggests that patients with EF have cytokine abnormalities similar to atopic patients, but with a striking elevation of transforming growth factor beta1. The responsiveness of the clinical symptoms and cytokine profile to low dose prednisone supports treatment with low dose immunosuppressive therapy in this disorder.

Topics: Anti-Inflammatory Agents; Arm; Child, Preschool; Cytokines; Eosinophilia; Eosinophils; Fasciitis; Female; Humans; Immunosuppressive Agents; Interleukin-5; Leukocyte Count; Prednisone; Transforming Growth Factor beta

To compare the expression of the genes encoding transforming growth factor-beta1 (TCF-beta1) and several extracellular matrix proteins between fascial and dermal fibroblasts from 3 patients with diffuse fasciitis with eosinophilia (DFE) of recent onset.. Fibroblasts were separately cultured from the fascia and dermis from each patient. Collagen production and mRNA levels for fibronectin, alpha1(I) procollagen, alpha1(III) procollagen. and the 3 chains of type VI collagen were compared between fascial and dermal fibroblasts from the same patient using biosynthetic studies with 14C-proline and Northern and dot blot hybridizations with specific cDNA. The expression of the gene encoding TGF-beta1 was also examined in these cultures by Northern hybridizations with human TGF-beta1 cDNA.. Fascial fibroblasts displayed 1.75 to 4.6-fold greater collagen biosynthesis, 3.4 to 8.5-fold elevation of the steady state levels of fibronectin mRNA, up to 3.9-fold elevation of the steady state mRNA levels for alpha1(I), alpha1(III) and alpha3(VI) collagens, and a marked increase in TGF-beta1 mRNA levels compared to fibroblasts from the adjacent dermis from the same individuals.. The expression of genes encoding several extracellular matrix proteins is increased in fascial fibroblasts from patients with diffuse fasciitis compared to fibroblasts from the adjacent dermis of the same individuals. The elevation of TGF-beta1 mRNA in the fascial cells indicates that this growth factor may play an important role in the pathogenesis of fibrosis in DFE.

Topics: Blotting, Northern; Cells, Cultured; Collagen; Eosinophilia; Fascia; Fasciitis; Fibroblasts; Fibronectins; Fibrosis; Gene Expression Regulation; Humans; RNA, Messenger; Skin; Transforming Growth Factor beta

Full-thickness skin biopsies obtained from four patients with rapidly progressive diffuse fasciitis associated with the Eosinophilia-Myalgia syndrome (EMS) were examined for the expression of transforming growth factor-beta 1 (TGF-beta 1), type VI collagen, and fibronectin genes employing immunohistochemistry and in situ hybridizations. The immunohistochemical studies demonstrated increased deposition of TGF-beta, type VI collagen, and fibronectin epitopes in the extracellular matrix of the fascia in comparison to the adjacent dermis in the same specimens. Increased levels of type VI collagen mRNA, as evidenced by positive in situ hybridization signals with an alpha 2(VI) collagen cDNA, were also found in the fascia in comparison with the dermis. In situ hybridizations of affected fascia with a human sequence-specific TGF-beta 1 cDNA demonstrated numerous fibroblasts displaying positive hybridization signals indicative of high levels of transcripts for this cytokine. In contrast, no hybridization signal for TGF-beta 1 was detected in fibroblasts in the adjacent dermis. These findings suggest that TGF-beta 1 may play an important role in the development of the connective tissue alterations present in EMS-associated diffuse fasciitis.

Topics: Aged; Collagen; Eosinophilia; Epitopes; Fasciitis; Female; Fibronectins; Fluorescent Antibody Technique; Gene Expression; Humans; Male; Middle Aged; Muscles; Nucleic Acid Hybridization; Pain; Skin; Syndrome; Transforming Growth Factor beta; Tryptophan