Pirmez Rodrigo, Barreto Taynara, Duque-Estrada Bruna, Quintella Danielle C, Cuzzi Tullia
Department of Dermatology, Instituto de Dermatologia Professor Rubem David Azulay, Santa Casa da Misericórdia do Rio de Janeiro, Rio de Janeiro, Brazil.
Skin Appendage Disord. 2018 Aug;4(3):145-149. doi: 10.1159/000481695. Epub 2017 Nov 4.
Frontal fibrosing alopecia (FFA) is considered a variant of lichen planopilaris affecting mainly the frontotemporal hairline. Since the first report in 1994, several other clinical features have been associated with the disease, such as facial papules (FP). Even though FP have been linked to facial vellus hair follicle involvement, how this finding alone could lead to the formation of clinically evident FP in FFA patients had not yet been addressed.
To describe histopathological findings of FP in the context of FFA and to highlight features that may be linked to their clinical formation.
Cutaneous FP biopsies of FFA patients performed between January 2016 and May 2017 were retrieved from our pathology database and reexamined by 2 pathologists.
Histological sections of thirteen 3.0-mm punch biopsy specimens (2 horizontally and 11 vertically oriented) were collected from 7 patients. Eleven specimens demonstrated prominent sebaceous glands and 10 dilated sebaceous ducts. Pinkus acid orcein staining revealed reduction and fragmentation of the elastic fibers in 12 samples and, in 7 of these, this finding was observed in both the papillary and reticular dermis, particularly around sebaceous lobules. Vellus hair follicle involvement was only seen in 2 samples.
Prominent sebaceous lobules with dilated ducts associated with an abnormal elastic framework seem to be the main explanation for the formation of FP in the context of FFA.
额部纤维性脱发(FFA)被认为是扁平苔藓性毛发角化病的一种变体,主要影响额颞部发际线。自1994年首次报道以来,该疾病还出现了其他一些临床特征,如面部丘疹(FP)。尽管FP与面部毳毛毛囊受累有关,但仅这一发现如何导致FFA患者出现临床上明显的FP尚未得到解答。
描述FFA背景下FP的组织病理学表现,并突出可能与其临床形成相关的特征。
从我们的病理数据库中检索2016年1月至2017年5月期间对FFA患者进行的皮肤FP活检标本,并由2名病理学家重新检查。
从7例患者中收集了13个3.0毫米钻孔活检标本的组织学切片(2个水平方向和11个垂直方向)。11个标本显示皮脂腺突出,10个标本显示皮脂腺导管扩张。平卡斯酸性地衣红染色显示12个样本中的弹性纤维减少和断裂,其中7个样本在乳头层和网状层均观察到这一发现,尤其是在皮脂腺小叶周围。仅在2个样本中观察到毳毛毛囊受累。
皮脂腺小叶突出伴导管扩张以及弹性框架异常似乎是FFA背景下FP形成的主要原因。