Increased Serum Levels of Interleukin-6, Immunoglobulin and Acute Phase Protein in Patients with the Severe Clinical Form of Inherited Epidermolysis Bullosa

Kawakami, Yoshio; Oyama, Noritaka; Ohtsuka, Mikio; Nakamura, Koichiro; Kaneko, Fumio

doi:10.1111/j.1346-8138.2005.tb00790.x

Cited by 10 publications

(11 citation statements)

References 13 publications

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“…According to previously reported data, 12 we found high IL-6 serum levels in DEB patients. This cytokine, mainly produced by activated macrophages, monocytes, antigen-presenting cells, and lymphocytes, is capable of promoting Th17 cell and monocyte differentiation, and also inducing the production of acute-phase proteins, thus playing a key role in the switch from innate to acquired immunity.…”

Section: Discussionsupporting

confidence: 83%

Proinflammatory Cytokines and Antiskin Autoantibodies in Patients With Inherited Epidermolysis Bullosa

et al. 2015

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Epidermolysis bullosa (EB) is a rare disorder characterized by inherited skin adhesion defects with abnormal disruption of the epidermal–dermal junction in response to mechanical trauma. Our aim was to investigate a set of cytokine levels in serum samples from patients suffering from epidermolysis bullosa simplex (EBS), dystrophic epidermolysis bullosa (DEB), and healthy controls (HCs), exploring their potential correlations with antiskin autoantibody titers and disease activity. Forty patients afferent to the Dermatological Ward of Bari City Hospital and 9 HCs were enrolled and subdivided according to the dystrophic (DEB) and simplex forms (EBS). We found a significant increase in interleukin (IL)-1β plasmatic levels of DEB (P = 0.0224) and EBS (P = 0.0465) patients compared to HCs; IL-6 levels were significantly higher in DEB than in EBS patients (P = 0.0004) or HCs (P = 0.0474); IL-2 levels were significantly increased in DEB compared with EBS (P = 0.0428). Plasmatic tumor necrosis factor-β and interferon-γ were higher in DEB patients than in HCs (P = 0.0448 and 0.0229). Conversely, tumor necrosis factor-α was significantly decreased in DEB (P = 0.0034). IL-5 correlated with anti-BP180 (r = −0.5018, P = 0.0338), anti-BP230 (r = −0.6097, P = 0.0122), and anticollagen VII (r = −0.5166, P = 0.0405) autoantibodies; interferon-γ correlated with anti-BP180 (r = 0.9633, P < 0.0001), anti-BP230 (r = 0.9071, P < 0.0001), and anticollagen VII (r = 0.8619, P = 0.0045) autoantibodies. Score of disease severity was significantly correlated with IL-6 (r = 0.6941, P = 0.029) and IL-12 (r = 0.5503, P = 0.0272). The present study supports that EB might be considered a systemic inflammatory disease rather than a skin-limited disorder; clinical disease activity scores could be also integrated by laboratory data such as IL-6 and IL-12 dosage; biotherapies targeting specific cytokine networks probably represent a way to go in the future.

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Section: Discussionsupporting

confidence: 83%

Proinflammatory Cytokines and Antiskin Autoantibodies in Patients With Inherited Epidermolysis Bullosa

et al. 2015

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“…Interestingly, IFN-γ has been reported to boost either the expression of HLA DQ2/8 or of transglutaminase 2 amplifying the responsivity to gluten peptides [27,28]. Indeed, increased serum levels of interleukin-6, immunoglobulin and acute phase protein have been reported in patients with the severe clinical form of EB [29].…”

Section: Resultsmentioning

confidence: 99%

Hla Typing in Epidermolysis Bullosa Patients: Relevancy to Gluten Sensitivity

Annicchiarico¹,

MG²

2013

J Genet Syndr Gene Ther

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“…IL-6, mainly produced by activated macrophages, lymphocytes, and fibroblasts, is well known to be a proinflammatory cytokine with pleiotropic functions and induce the production of acute-phase proteins, inducing systemic fever and C-reactive protein and immunoglobulin production. 55,56 Although the etiological relevance of IL-6 in EB patients with fever is unknown, there are potentially sufficient prospective data to understand the nature of its action in inflammatory skin diseases, such as generalized pustular psoriasis, palmoplantar pustulosis, and acute urticaria. 55,[57][58][59] Kawakami et al 55 reported that all the sera from their five unrelated patients with EB had markedly increased IL-6 levels.…”

Section: Ta B L E 2 (Continued)mentioning

confidence: 99%

“…55,56 Although the etiological relevance of IL-6 in EB patients with fever is unknown, there are potentially sufficient prospective data to understand the nature of its action in inflammatory skin diseases, such as generalized pustular psoriasis, palmoplantar pustulosis, and acute urticaria. 55,[57][58][59] Kawakami et al 55 reported that all the sera from their five unrelated patients with EB had markedly increased IL-6 levels. In addition, Odorisio et al 60 reported a case of RDEB in which the expression of IL-6 was upregulated in a twin with a more severe phenotype.…”

Section: Ta B L E 2 (Continued)mentioning

confidence: 99%

New insight of itch mediators and proinflammatory cytokines in epidermolysis bullosa

Nguyen

Shinkuma

Katsumi

et al. 2022

J Cutaneous Imm & Allergy

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Objectives Epidermolysis bullosa (EB) is a hereditary disorder characterized by mechanical stress‐induced blistering. The presence of extracutaneous complications such as cardiomyopathy and renal disease observed in severe EB subtypes and the fact that pruritus is a common symptom across all EB subtypes indicate that EB is not only a skin fragility disease but also a systemic inflammatory disorder. Our study aims to elucidate the basis of the systemic inflammation seen in EB patients. Methods We analyzed serum samples of 20 EB patients by Luminex bead‐based cytokine assays and enzyme‐linked immunosorbent assays. Results The serum levels of sIL‐2R, IL‐6, HGF, M‐CSF, SCGF‐β, IL‐8, IL‐16, IFN‐γ, MIF, MIP‐1α, and thymic stromal lymphopoietin (TSLP) (p < .01, p < .01, p < .01, p < .01, p < .01, p < .05, p < .05, p < .05, p < .05, p < .05, and p = .01, respectively) were found to be significantly elevated in EB patients, whereas TNF‐β (p < .01) was decreased. Th2 cytokines including IL‐4, IL‐5, and IL‐13 were not elevated in EB patients. In contrast, TSLP was significantly increased, and IL‐31 and oncostatin M were not statistically significant but tended to be higher in the patients than in healthy controls. Among proinflammatory cytokines such as IL‐1β, IL‐6, and TNF‐α, IL‐6 was elevated in EB patients. Conclusions The imbalance of several itch mediators and proinflammatory cytokines was identified. Biologics targeting the cytokines found to be elevated in the sera of patients is considered as a beneficial treatment option for EB.

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Increased Serum Levels of Interleukin-6, Immunoglobulin and Acute Phase Protein in Patients with the Severe Clinical Form of Inherited Epidermolysis Bullosa

Cited by 10 publications

References 13 publications

Proinflammatory Cytokines and Antiskin Autoantibodies in Patients With Inherited Epidermolysis Bullosa

Proinflammatory Cytokines and Antiskin Autoantibodies in Patients With Inherited Epidermolysis Bullosa

Hla Typing in Epidermolysis Bullosa Patients: Relevancy to Gluten Sensitivity

New insight of itch mediators and proinflammatory cytokines in epidermolysis bullosa

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