Matheo Augusto Morandi Stumpf, MD, Av. Carlos Cavalcanti, 4748, Medicine Department, Uvaranas, CEP: 84030-900, Ponta Grossa, Brazil;
Acta Dermatovenerol Croat. 2020 Dec;28(3):188-189.
A 53 year-old-woman presented to our dermatology clinic with a 3-year history of hair loss and pruritus of the anterior scalp. She denied drug use, comorbidities, and other systemic symptoms. Physical examination revealed a band-like recession of the frontotemporal hairline with loss of the eyebrows and perifollicular erythema. The alopecic skin was atrophic and lighter than the chronically sun-exposed forehead. Some "lonely hairs" were observed, as well as depression of temporal veins. Systemic examination was unremarkable. A diagnosis of frontal fibrosing alopecia (FFA) was established, no biopsy was performed, and treatment with hydroxychloroquine 400 mg/daily with topical minoxidil was promptly started. After 6 months, the patient stopped the drugs because no clinical improvement was observed. More than one year later, she returned with the same symptoms demanding new treatment. We opted to change the immunosuppressant/immunomodulator to methotrexate monotherapy 20 mg once a week to improve adherence. Patient achieved clinical stabilization within seven months of treatment. She maintained with the same clinical features as described above but had an eyebrow tattoo made, and perifollicular erythema was no longer present (Figure 1). FFA is a cicatricial alopecia caused by immune-mediated inflammatory infiltrate lymphocytes in the infundibulo-isthmic region of the hair follicle. Its etiopathogenesis is unknown, and it usually occurs in postmenopausal Caucasian women. Clinically, FFA presents with progressive loss of frontotemporal hairline resulting in a lighter skin compared with the forehead and with absence of follicular ostia. Perifollicular erythema, pruritus, pain, the "lonely hair sign", and partial or total scarring eyebrow loss can be encountered as well. Biopsy is no longer necessary, but if it is done it is important to remember that there are no pathological criteria, at present, to distinguish FFA from lichen planopilaris (1,2). There have been no randomized clinical trial on AFF treatment. Topical drugs such as corticosteroids, minoxidil, and calcineurin inhibitors as well as systemic treatments such as 5α-reductase inhibitors, hydroxychloroquine, retinoids, and methotrexate can usually be used (1,2). The Frontal Fibrosing Alopecia Severity Score should be used during treatment (3), especially regarding the search for perifollicular erythema, which is well-known for having a direct correlation with progressive disease (4). In a retrospective study with 19 patients at Duke University, methotrexate monotherapy achieved stabilization in one of two patients. The mean duration of therapy was 16 months, and the dose varied between 15-25 mg once a week (5). Unfortunately, the variable course of this disease and the possibility of spontaneous stabilization lead to risk of overestimating the effects of the prescribed treatments (1). After clinical stabilization, drug withdrawal could be attempted with frequent clinical observation. This difficulty persists as no patient is likely to agree with that.
一位 53 岁女性因脱发和前头皮瘙痒 3 年到皮肤科就诊。她否认药物使用、合并症和其他全身症状。体格检查显示额颞部发际线呈带状退缩,眉毛缺失,毛囊周围红斑。脱发皮肤萎缩,比慢性日晒的额头颜色更浅。观察到一些“孤独的毛发”,以及颞静脉凹陷。全身检查无异常。诊断为额部纤维性脱发(FFA),未行活检,立即开始每日 400mg 羟氯喹联合外用米诺地尔治疗。6 个月后,患者因未观察到临床改善而停药。一年多后,她因同样的症状返回要求新的治疗。我们选择将免疫抑制剂/免疫调节剂改为每周一次 20mg 甲氨蝶呤单药治疗,以提高依从性。治疗 7 个月后患者达到临床稳定。她保持了上述相同的临床特征,但纹了眉毛,毛囊周围红斑不再存在(图 1)。FFA 是一种瘢痕性脱发,由毛囊漏斗部-峡部的免疫介导的炎症浸润淋巴细胞引起。其病因不明,通常发生在绝经后白种女性中。临床上,FFA 表现为额颞部发际线进行性丧失,导致与额头相比皮肤颜色更浅,且没有毛囊口。也可能出现毛囊周围红斑、瘙痒、疼痛、“孤独毛发”和部分或全部瘢痕性眉毛缺失。活检已不再必要,但如果进行活检,重要的是要记住,目前尚无病理标准可将 FFA 与扁平苔藓样瘢痕性脱发(lichen planopilaris,LPP)区分开来(1,2)。目前尚无 AFF 治疗的随机临床试验。局部药物如皮质类固醇、米诺地尔和钙调磷酸酶抑制剂,以及全身治疗如 5α-还原酶抑制剂、羟氯喹、类视黄醇和甲氨蝶呤通常可用于治疗(1,2)。在治疗过程中应使用额部纤维性脱发严重程度评分(3),特别是寻找毛囊周围红斑,这与疾病进展有直接相关性(4)。在杜克大学的一项回顾性研究中,19 名患者中,有 1 名接受甲氨蝶呤单药治疗后病情稳定。平均治疗时间为 16 个月,剂量为每周 15-25mg(5)。不幸的是,这种疾病的多变过程和自发稳定的可能性导致了对所开治疗效果的高估(1)。在临床稳定后,可以尝试在密切临床观察下停药。由于没有患者可能同意,所以这仍然是一个困难。