Osuoji Olive C, DeGrazia Taryn, Rolader Robin, Cole Emily F, Lawson Katy, Hilley Henry, Xing Yanyan, Han Liang, Chisolm Sarah, Claussen Henry, Yan Xiaobo, Sun Yuxian, Liu Yuan, Chen Suephy C, Feldman Ron J
Department of Dermatology, Emory University School of Medicine, Atlanta, Georgia, USA.
Department of Biostatistics and Bioinformatics, Emory University Rollins School of Public Health, Atlanta, Georgia, USA.
JID Innov. 2024 Nov 21;5(2):100329. doi: 10.1016/j.xjidi.2024.100329. eCollection 2025 Mar.
Bullous pemphigoid is a devastating autoimmune blistering disease with need for improved therapeutics. Limited data are available on the overall burden of pruritus and alterations over time; however, treatment of itch-specific pathways may provide novel therapeutics. In this paper, we analyzed the QOL impact particularly related to itch and determined corresponding changes in intraepidermal nerve fiber density and gene expression. A total of 43 patients with bullous pemphigoid were followed prospectively on standard-of-care treatment and showed average Bullous Pemphigoid Disease Area Index total activity score decrease from 19.1 ± 19.2 to 8.2 ± 11.3 and improvement in QOL measures Autoimmune Bullous Disease Quality of Life, Treatment of Autoimmune Bullous Disease Quality of Life, and ItchyQoL. At baseline, intraepidermal nerve fiber density in patients with bullous pemphigoid and atopic dermatitis were significantly lower than in healthy controls (7.3 ± 1.5 and 3.2 ± 2.0 vs 9.7 ± 5.4 fibers/mm, = .031) and increased from baseline to follow-up visit (11.7 ± 0.4 and 5.8 ± 2.7), although only atopic dermatitis reached statistical significance ( = .018). , , , and genes were highly upregulated in the skin of patients with bullous pemphigoid compared with those in healthy controls. Our data provide evidence for improvements in itch-related QOL over time on standard-of-care therapies with unique alterations in inflammatory mediators related to early immune cell activation and recruitment.
大疱性类天疱疮是一种严重的自身免疫性水疱病，需要改进治疗方法。关于瘙痒的总体负担及其随时间的变化，现有数据有限；然而，针对瘙痒特异性途径的治疗可能会提供新的治疗方法。在本文中，我们分析了与瘙痒特别相关的生活质量影响，并确定了表皮内神经纤维密度和基因表达的相应变化。共有43例大疱性类天疱疮患者接受了标准治疗的前瞻性随访，结果显示大疱性类天疱疮疾病面积指数总活动评分平均从19.1±19.2降至8.2±11.3，生活质量指标自身免疫性大疱病生活质量、自身免疫性大疱病治疗生活质量和瘙痒生活质量均有所改善。基线时，大疱性类天疱疮患者和特应性皮炎患者的表皮内神经纤维密度显著低于健康对照者（分别为7.3±1.5和3.2±2.0，而健康对照者为9.7±5.4条纤维/mm，P = 0.031），且从基线到随访时有所增加（分别为11.7±0.4和5.8±2.7），尽管只有特应性皮炎达到统计学显著性（P = 0.018）。与健康对照者相比，大疱性类天疱疮患者皮肤中的[具体基因名称未给出]、[具体基因名称未给出]、[具体基因名称未给出]和[具体基因名称未给出]基因高度上调。我们的数据为在标准治疗下，与早期免疫细胞激活和募集相关的炎症介质发生独特变化，随时间瘙痒相关生活质量得到改善提供了证据。
