National Reference Centre for Rare Skin Diseases, Hôpital St André, CHU de Bordeaux, France.
Br J Dermatol. 2014 Apr;170(4):816-23. doi: 10.1111/bjd.12691.
Polymorphisms of NLR (nucleotide-binding domain and leucine rich repeat containing) family, pyrin domain containing protein 1 (NLRP1) have been found in patients with vitiligo/nonsegmental vitiligo (NSV), and increased NLRP1 expression has been detected in the leading edge of lesional skin biopsies.
To evaluate the presence and intensity of NLRP1 immunostaining in lesional and perilesional skin of patients with vitiligo/NSV and to search for possible correlations between NLRP1 and interleukin (IL)-1β expression, lymphocytic infiltrates and disease activity.
Of 14 consecutive vitiligo/NSV patients, eight had active disease [Vitiligo European Task Force (VETF) spreading score +1 to +5], one patient had stable disease and five patients had regressive disease (VETF spreading score -1 to -3). We performed immunostaining for NLRP1, B and T lymphocytes, IL-1β and kallikrein 7 on lesional and perilesional vitiligo skin.
NLRP1 and IL-1β immunostaining in perilesional vitiligo/NSV skin was significantly associated with progressive disease (P = 0·009 and 0·04, respectively) and performed better than the simple detection of lymphocytic infiltrates.
Our findings suggest that markers of the NLRP1 inflammasome could be a useful test for assessing disease activity in addition to the detection of inflammatory infiltrates in the progressing margins of vitiligo/NSV lesions.
核苷酸结合域富含亮氨酸重复蛋白(NLR)家族和富含吡咯啉结构域蛋白 1(NLRP1)的多态性已在白癜风/非节段性白癜风(NSV)患者中被发现,并且在病变皮肤活检的前沿部位检测到 NLRP1 表达增加。
评估白癜风/NSV 患者皮损和皮损周围皮肤中 NLRP1 免疫染色的存在和强度,并寻找 NLRP1 与白细胞介素(IL)-1β表达、淋巴细胞浸润和疾病活动之间的可能相关性。
对 14 例连续的白癜风/NSV 患者进行研究,其中 8 例为活动性疾病(白癜风欧洲工作组(VETF)扩散评分+1 至+5),1 例为稳定期疾病,5 例为退行性疾病(VETF 扩散评分-1 至-3)。我们对白癜风/NSV 皮损和皮损周围皮肤进行 NLRP1、B 和 T 淋巴细胞、IL-1β 和激肽 7 的免疫染色。
皮损周围白癜风/NSV 皮肤中的 NLRP1 和 IL-1β 免疫染色与进行性疾病显著相关(P=0.009 和 0.04),且比简单检测淋巴细胞浸润更有意义。
我们的研究结果表明,NLRP1 炎症小体的标志物除了可以检测白癜风/NSV 病变进展边缘的炎症浸润外,还可以作为评估疾病活动的有用指标。