Immunological mechanisms underlying progression of chronic wounds in recessive dystrophic epidermolysis bullosa.
Immunological mechanisms underlying progression of chronic wounds in recessive dystrophic epidermolysis bullosa.
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DOI：
10.1111/exd.14411
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发表时间：
2021-12
影响因子：
3.6
通讯作者：
中科院分区：
文献类型：
--
作者：
遗传性大疱性表皮病（EB）是一种机械性大疱性皮肤脆性疾病，其特征是上皮粘附缺陷，导致机械应力诱导的皮肤起泡。根据真皮-表皮交界处的组织分离程度，EB分为单纯型（EBS）、交界型（JEB）、营养不良型（DEB）和Kindler综合征。EB无法治愈，疼痛的慢性皮肤伤口是隐性（RDEB）患者的主要并发症之一。虽然RDEB被认为是一种皮肤疾病，但最近的数据支持潜在的全身免疫缺陷。此外，慢性伤口通常定植有致病性微生物群，导致过度炎症和伤口愈合改变。因此，患有RDEB的患者遭受慢性皮肤瘙痒/烧灼的疼痛感以及与细菌感染的无休止的战斗。为了提高他们的生活质量和预期寿命，预防皮肤感染，抑制慢性炎症和刺激伤口愈合非常重要。对RDEB患者慢性愈合不良伤口维持的免疫学事件有明确的科学认识，这对于改善疾病管理和更好地了解其他伤口愈合障碍是必要的。在这篇综述中，我们总结了目前对专业吞噬细胞（如中性粒细胞，巨噬细胞和树突状细胞）的作用，T细胞介导的免疫在淋巴器官中的作用，以及微生物群与RDEB伤口愈合不良的关系的认识。我们的结论是，RDEB患者有一个潜在的免疫缺陷，似乎影响抗菌免疫。
Hereditary epidermolysis bullosa (EB) is a mechanobullous skin fragility disorder characterized by defective epithelial adhesion, leading to mechanical stress‐induced skin blistering. Based on the level of tissue separation within the dermal‐epidermal junction, EB is categorized into simplex (EBS), junctional (JEB), dystrophic (DEB) and Kindler syndrome. There is no cure for EB, and painful chronic cutaneous wounds are one of the major complications in recessive (RDEB) patients. Although RDEB is considered a cutaneous disease, recent data support the underlying systemic immunological defects. Furthermore, chronic wounds are often colonized with pathogenic microbiota, leading to excessive inflammation and altered wound healing. Consequently, patients with RDEB suffer from a painful sensation of chronic, cutaneous itching/burning and an endless battle with bacterial infections. To improve their quality of life and life expectancy, it is important to prevent cutaneous infections, dampen chronic inflammation and stimulate wound healing. A clear scientific understanding of the immunological events underlying the maintenance of chronic poorly healing wounds in RDEB patients is necessary to improve disease management and better understand other wound healing disorders. In this review, we summarize current knowledge of the role of professional phagocytes, such as neutrophils, macrophages and dendritic cells, the role of T‐cell‐mediated immunity in lymphoid organs, and the association of microbiota with poor wound healing in RDEB. We conclude that RDEB patients have an underlying immunity defect that seems to affect antibacterial immunity.
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DOI：
10.1242/dev.087320
发表时间：
2013-05
期刊：
Development (Cambridge, England)
影响因子：
--
作者：
Farley AM;Morris LX;Vroegindeweij E;Depreter ML;Vaidya H;Stenhouse FH;Tomlinson SR;Anderson RA;Cupedo T;Cornelissen JJ;Blackburn CC
通讯作者：
Blackburn CC
影响因子：
5.2
作者：
Asadollahi P;Farahani NN;Mirzaii M;Khoramrooz SS;van Belkum A;Asadollahi K;Dadashi M;Darban-Sarokhalil D
通讯作者：
Darban-Sarokhalil D
影响因子：
6.9
作者：
Has, Cristina;Nystroem, Alexander;Uitto, Jouni
通讯作者：
Uitto, Jouni
DOI：
10.1038/nri3567
发表时间：
2014-01
期刊：
Nature reviews. Immunology
影响因子：
--
作者：
通讯作者：
--
影响因子：
3.6
作者：
Huitema L;Phillips T;Alexeev V;Tomic-Canic M;Pastar I;Igoucheva O
通讯作者：
Igoucheva O