Section of Dermatology, Department of Health Sciences, University of Florence, Florence, Italy.
Experimental Immunology Laboratory, Istituto Dermopatico dell'Immacolata (IDI-IRCCS), Rome, Italy.
Exp Dermatol. 2023 Jun;32(6):915-921. doi: 10.1111/exd.14791. Epub 2023 Mar 20.
Bullous pemphigoid (BP) is an autoimmune bullous disease, characterized by autoantibodies targeting BP180 and BP230. The role of interleukin (IL)-36, a potent chemoattractant for granulocytes, in BP remains elusive.The expression of IL-36 cytokines (IL-36α, β, γ) and their antagonists (IL-36Ra and IL-38) was analysed in the skin and serum samples of patients with BP (n = 31), psoriasis (n = 10) and healthy controls (HC) (n = 14) by quantitative polymerase chain reaction and enzyme linked immunosorbent assay, respectively. Skin and serum levels of all cytokines were correlated with the Bullous Pemphigoid Disease Area Index (BPDAI) score and with the serum concentration of pathogenic antibodies.IL-36α, IL-36β, IL-36γ and IL-36Ra were significantly (p < 0.05) overexpressed in BP skin compared to HC, without remarkable differences relative to psoriasis skin. The expression of IL-38 was significantly (p < 0.05) higher in BP compared to psoriasis skin.IL-36α and γ, but not β, serum concentrations were significantly (p < 0.05) higher in BP compared to HC. IL-36γ was significantly (p < 0.05) more expressed in the serum of psoriasis patients than BP. The serum concentration of IL-36Ra and IL-38 were similar between BP and HC, while IL-38 serum levels were significantly (p < 0.05) higher in BP compared to psoriasis patients. Serum IL-36α correlated significantly with BPDAI (r = 0.5 p = 0.001).IL-36 agonists are increased in BP patients, both locally and systemically. Serum IL-36α might represent a potential biomarker for BP. An inefficient balance between IL-36 agonists and antagonists is likely to occur during BP inflammation.
大疱性类天疱疮（BP）是一种自身免疫性大疱性疾病，其特征是针对 BP180 和 BP230 的自身抗体。白细胞介素（IL）-36，一种有效的粒细胞趋化因子，在 BP 中的作用仍不清楚。通过定量聚合酶链反应和酶联免疫吸附试验分别分析了 BP（n=31）、银屑病（n=10）和健康对照（HC）（n=14）患者皮肤和血清样本中 IL-36 细胞因子（IL-36α、β、γ）及其拮抗剂（IL-36Ra 和 IL-38）的表达。皮肤和血清中所有细胞因子的水平均与大疱性类天疱疮疾病面积指数（BPDAI）评分以及致病抗体的血清浓度相关。与 HC 相比，BP 皮肤中 IL-36α、IL-36β、IL-36γ 和 IL-36Ra 的表达显著（p<0.05）增加，但与银屑病皮肤相比无明显差异。与银屑病皮肤相比，BP 皮肤中 IL-38 的表达显著（p<0.05）增加。与 HC 相比，BP 血清中 IL-36α 和 γ（但不是β）的浓度显著（p<0.05）增加。与 BP 相比，银屑病患者血清中 IL-36γ 的表达显著（p<0.05）更高。BP 和 HC 之间血清 IL-36Ra 和 IL-38 的浓度相似，而 BP 血清中 IL-38 的水平显著（p<0.05）高于银屑病患者。血清 IL-36α 与 BPDAI 显著相关（r=0.5，p=0.001）。BP 患者体内局部和系统均增加了 IL-36 激动剂。血清 IL-36α 可能是 BP 的潜在生物标志物。在 BP 炎症过程中，IL-36 激动剂和拮抗剂之间可能存在不平衡。
