Harries Matthew J, Meyer Katja C, Paus Ralf
Dermatological Sciences, The University of Manchester, Manchester, UK.
Autoimmun Rev. 2009 May;8(6):478-83. doi: 10.1016/j.autrev.2008.09.003. Epub 2008 Oct 14.
Primary cicatricial alopecias (PCA) represent uncommon inflammatory disorders that result in permanent loss of scalp hair. Cutaneous autoimmunity, most prominently chronic cutaneous lupus erythematosus (CCLE), can result in this kind of scarring hair loss. The cosmetic disfigurement caused by PCA and the very unsatisfactory therapeutic options available to date all demand a better understanding of the obscure pathobiology of PCA so as to define new therapeutic targets and strategies. Hair follicle (HF) cycling and regeneration are abolished in PCA due to irreversible, epithelial hair follicle stem cell (eHFSC) damage, triggered by major, yet unclear pro-inflammatory events (e.g. type I interferon-associated cytotoxic inflammation, loss of HF immune privilege, loss of immunosuppressive "no danger" signals). Therefore, immuno-protection of eHFSC and restitution of their immune privilege are attractive future therapeutic strategies in PCA. Chronic cutaneous lupus erythematosus-associated PCA may serve as a model system for other diseases where epithelial stem cells undergo immuno-destruction.
原发性瘢痕性脱发（PCA）是一类罕见的炎症性疾病，可导致头皮永久性脱发。皮肤自身免疫，最显著的是慢性皮肤红斑狼疮（CCLE），可导致这种瘢痕性脱发。PCA造成的美容缺陷以及目前可用的治疗选择非常不理想，这一切都需要更好地理解PCA obscure的病理生物学，以确定新的治疗靶点和策略。由于主要但尚不清楚的促炎事件（如I型干扰素相关的细胞毒性炎症、毛囊免疫特权丧失、免疫抑制“无危险”信号丧失）引发的不可逆上皮毛囊干细胞（eHFSC）损伤，PCA中毛囊（HF）的周期性生长和再生被消除。因此，eHFSC的免疫保护及其免疫特权的恢复是PCA未来有吸引力的治疗策略。慢性皮肤红斑狼疮相关的PCA可作为上皮干细胞发生免疫破坏的其他疾病的模型系统。
