Ma Sophia A, Imadojemu Sotonye, Beer Kenneth, Seykora John T
Department of Pathology and Laboratory Medicine, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania.
Department of Dermatology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania.
J Cutan Pathol. 2017 Aug;44(8):672-676. doi: 10.1111/cup.12955. Epub 2017 Jun 1.
Frontal fibrosing alopecia (FFA) is a cicatricial alopecia typically occurring in postmenopausal women. The etiology and pathophysiology of FFA is poorly understood but thought to be immune mediated. This study aims to further explore the extent of fibrosis and the inflammatory microenvironment by characterizing Langerhans cells (LCs), helper T cells, cytotoxic T cells and B cells near hair follicles in FFA.
Eleven paraffin-embedded tissues from patients with a clinical and histopathologic diagnosis of FFA were selected for immunohistochemical studies using CD3, CD4, CD8, CD1a and CD20. The lymphocytes and LCs were counted around involved follicles. The CD4/CD8 T-lymphocyte ratios were calculated and compared to the CD4/CD8 T-lymphocyte ratios in uninvolved areas.
On histopathologic review, at least 35% of follicles in each case were affected by the disease with concentric perifollicular fibrosis and a perifollicular lichenoid lymphocytic infiltrate around the infundibuloisthmic portion of the hair follicle. There was an increase of perifollicular LCs (mean of 18, SD of 5.5) and intrafollicular LCs (mean of 14, SD of 4.3) in involved follicles compared to uninvolved follicles (P < .0001). The involved follicles also showed a relative decrease in the CD4/CD8 ratio indicating increased numbers of CD8+ T cells; a finding distinct from the CD4-predominant population in uninvolved follicles (P < .0001).
The inflammatory features of FFA show a CD8-biased T-cell infiltrate with increased numbers of LCs in the infundibuloisthmic region. The increased LCs may represent an aberrant immune reaction promoting a CD8+ T-cell response.
额部纤维性秃发（FFA）是一种瘢痕性秃发，通常发生于绝经后女性。FFA的病因和病理生理学尚不清楚，但被认为是免疫介导的。本研究旨在通过对FFA患者毛囊周围的朗格汉斯细胞（LCs）、辅助性T细胞、细胞毒性T细胞和B细胞进行特征分析，进一步探究纤维化程度和炎症微环境。
选取11例临床及组织病理学诊断为FFA的石蜡包埋组织，采用CD3、CD4、CD8、CD1a和CD20进行免疫组织化学研究。对受累毛囊周围的淋巴细胞和LCs进行计数。计算CD4/CD8 T淋巴细胞比值，并与未受累区域的CD4/CD8 T淋巴细胞比值进行比较。
组织病理学检查显示，每例中至少35%的毛囊受疾病影响，伴有同心性毛囊周围纤维化以及毛囊漏斗峡部周围的毛囊周苔藓样淋巴细胞浸润。与未受累毛囊相比，受累毛囊周围的LCs（平均值为18，标准差为5.5）和毛囊内LCs（平均值为14，标准差为4.3）增多（P <.0001）。受累毛囊的CD4/CD8比值也相对降低，表明CD8+ T细胞数量增加；这一发现与未受累毛囊中以CD4为主的细胞群体不同（P <.0001）。
FFA的炎症特征表现为CD8偏向性T细胞浸润，漏斗峡部区域的LCs数量增加。LCs数量增加可能代表一种异常免疫反应，促进CD8+ T细胞反应。
