Scarring Alopecia in a 66-Year-Old Woman

Sergio Castillo Pinto, Lina M. Isaza, Isabel Cristina Cuellar
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Notably, the patient reported similar episodes in her twenties, which had resulted in cicatricial alopecia over large areas of the scalp. Considering the clinical findings and the diagnostic alternatives, an incisional biopsy was performed for histopathology, direct immunofluorescence (DIF), and the salt-split test (Figures 3 and 4).</p><p>Cicatricial alopecia refers to a group of disorders that cause permanent hair loss due to the destruction of hair follicles. It can be classified as primary, involving lymphocytic, neutrophilic, mixed, or nonspecific etiologies, or secondary, resulting from physical factors such as trauma, thermal or surgical damage. Among these causes, BPP is rarely found as a possible aetiology.</p><p>BPP is a rare autoimmune blistering disease considered a variant of mucous membrane pemphigoid (MMP). To date, 65 cases have been reported in the literature with histopathological confirmation and positive DIF [<span>1-3</span>]. 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引用次数: 0

Abstract

A 66-year-old female with no significant medical history, referred from a rural area, presented with a 1-week history of ocular pain associated with ipsilateral erosions and tense bullae involving the left scalp and periocular region. On examination, extensive areas of alopecia were observed on the majority of the scalp, with no visible follicular openings, along with erosions and tense bullae on the left side of the scalp and periocular area. No involvement of other body areas or mucous membranes was noted (Figures 1 and 2). Initially, the differential diagnosis included herpes zoster or lichen planus pemphigoides with Wolf's isotopic response. A comprehensive ocular examination by an ophthalmologist ruled out herpetic infection or other structural damage to the eye. Notably, the patient reported similar episodes in her twenties, which had resulted in cicatricial alopecia over large areas of the scalp. Considering the clinical findings and the diagnostic alternatives, an incisional biopsy was performed for histopathology, direct immunofluorescence (DIF), and the salt-split test (Figures 3 and 4).

Cicatricial alopecia refers to a group of disorders that cause permanent hair loss due to the destruction of hair follicles. It can be classified as primary, involving lymphocytic, neutrophilic, mixed, or nonspecific etiologies, or secondary, resulting from physical factors such as trauma, thermal or surgical damage. Among these causes, BPP is rarely found as a possible aetiology.

BPP is a rare autoimmune blistering disease considered a variant of mucous membrane pemphigoid (MMP). To date, 65 cases have been reported in the literature with histopathological confirmation and positive DIF [1-3]. BPP is characterised by chronic and recurrent subepidermal bullae that most commonly appear on the scalp and face, leading to secondary scarring and cicatricial alopecia. Mucous membrane involvement is reported in only about 22.2% of cases and tends to be mild [3-6].

Diagnosis is based on clinical and histopathological correlation, with findings of subepidermal blisters and superficial dermal infiltrate containing eosinophils, along with DIF showing IgG and C3 deposits along the BMZ [3, 4]. When available, serologic studies such as indirect immunofluorescence and ELISA can help to confirm the diagnosis by identifying autoantibodies with specific target antigens [7]. If serologic tests are not available, the salt-split test can be a useful tool in DIF [7]. This test helps determine whether antibodies are binding to the epidermal or dermal side of the BMZ, providing information about the possible targeted antigens, which is not possible with DIF alone [7]. The pattern of antibody deposits in the BMZ, along with the salt-split test, helps identify the specific antigens involved; distinguishing between n-serrated and u-serrated patterns, and determining whether binding occurs on the epidermal or dermal side of the BMZ [8, 9]. BP180 and BP230, the antigens commonly involved in BPP, are located in the epidermal side of the BMZ and exhibit an n-serrated pattern. In contrast, other bullous diseases, such as epidermolysis bullosa acquisita (EBA), involve collagen VII, which is found in the dermal side of the BMZ and displays u-serrated pattern [7-10]. This differentiation aids in diagnosis [3, 6, 8]. There has been controversy over whether BPP is a variant of cicatricial pemphigoid or EBA. Recently it has been established that when BP180 or BP230 is the target antigen, it is classified as BPP, whereas if collagen VII is affected, it is considered BPP-like EBA [3, 9]. In our case, antibodies were detected on the epidermal side of the BMZ in the salt-split-test, indicating that the antigens involved corresponded to BP-180 or BP-230.

For treatment, topical high-potency steroids are typically used as the first-line therapy. However, a multimodal approach may be necessary to achieve lesion improvement. Immunomodulatory and immunosuppressive therapies such as methotrexate, azathioprine, tetracycline, dapsone or cyclosporine have been described, offering an adequate safety profile and leading to complete remission of lesions [3]. In our case, initial treatment with prednisolone 0.5 mg/kg/day and topical clobetasol resulted in significant improvement of lesions. Maintenance therapy with oral methotrexate at 15 mg/week, along with gradual corticosteroid tapering, led to complete healing of the skin lesions, with no evidence of new lesions.

BPP should be considered as a differential diagnosis in cases of cicatricial alopecia. It is important to correlate the patient's medical history, enquire about recurrences, and review physical examination findings, as well as biopsy and DIF reports. If serologic autoantibody identification is not available, the salt-split test serves as a useful and cost-effective diagnostic tool. This is the third reported case of BPP in a Hispanic female patient.

All authors contributed to the manuscript conception, design, drafting and revision of the article. All authors read and approved the final manuscript. All authors take responsibility for the integrity of the data and the accuracy of the data.

The project was reviewed and approved by the Ethics and Research Committee of the hospital, in accordance with applicable ethical guidelines. All patients in this manuscript have given written informed consent for participation in the study and the use of their de-identified, anonymized, aggregated data and their case details (including photographs) for publication.

The authors declare no conflicts of interest.

66岁女性的瘢痕性脱发
66岁女性,无明显病史,来自农村地区,眼部疼痛1周,伴有同侧糜烂和紧张大疱累及左侧头皮和眼周区域。检查时,大部分头皮可见大面积脱发,未见毛囊开口,左侧头皮和眼周有糜烂和紧张的大疱。未发现其他身体部位或粘膜受累(图1和2)。最初,鉴别诊断包括带状疱疹或扁平苔藓类天疱疮伴Wolf同位素反应。眼科医生进行了全面的眼部检查,排除了疱疹感染或其他眼部结构损伤。值得注意的是,该患者在20多岁时报告了类似的情况,导致大面积头皮瘢痕性脱发。考虑到临床表现和诊断选择,我们进行了切口活检进行组织病理学检查、直接免疫荧光(DIF)和盐裂试验(图3和4)。瘢痕性脱发是指由于毛囊被破坏而导致永久性脱发的一组疾病。它可分为原发性,包括淋巴细胞性、中性粒细胞性、混合性或非特异性病因,或继发性,由外伤、热损伤或手术损伤等物理因素引起。在这些原因中,很少发现BPP是一种可能的病因。BPP是一种罕见的自身免疫性水疱疾病,被认为是粘膜类天疱疮(MMP)的一种变体。迄今为止,文献中报道了65例组织病理学确诊和DIF阳性病例[1-3]。BPP的特征是慢性和复发性表皮下大疱,最常见于头皮和面部,导致继发性瘢痕和瘢痕性脱发。据报道,仅22.2%的病例累及粘膜,且往往是轻度的[3-6]。诊断基于临床和组织病理学相关性,发现表皮下水泡和含有嗜酸性粒细胞的表皮浅层浸润,同时DIF显示IgG和C3沿BMZ沉积[3,4]。如有可能,血清学研究,如间接免疫荧光和ELISA可以通过识别具有特定靶抗原[7]的自身抗体来帮助确诊。如果血清学试验不可用,盐裂试验可以是一个有用的工具在DIF bb0。该测试有助于确定抗体是否与BMZ的表皮或真皮侧结合,提供有关可能的靶向抗原的信息,这是单独使用DIF无法做到的。抗体在BMZ中的沉积模式,以及盐裂试验,有助于识别所涉及的特定抗原;区分n-锯齿和u-锯齿模式,并确定结合是发生在BMZ的表皮侧还是真皮侧[8,9]。BP180和BP230是BPP中常见的抗原,位于BMZ的表皮侧,呈n锯齿状。相比之下,其他大疱性疾病,如获得性大疱性表皮松解症(EBA),涉及胶原VII,在BMZ真皮侧发现,呈u形锯齿状[7-10]。这种鉴别有助于诊断[3,6,8]。关于BPP是否是瘢痕性类天疱疮或EBA的变体一直存在争议。最近有研究表明,当BP180或BP230为靶抗原时,被归类为BPP,而如果影响到VII胶原,则被认为是BPP样EBA[3,9]。在我们的病例中,盐裂试验中在BMZ表皮侧检测到抗体,表明所涉及的抗原对应于BP-180或BP-230。对于治疗,局部高效类固醇通常被用作一线治疗。然而,可能需要采用多模式方法来改善病变。免疫调节和免疫抑制疗法如甲氨蝶呤、硫唑嘌呤、四环素、氨苯砜或环孢素已被描述,提供足够的安全性并导致病变完全缓解。在我们的病例中,最初使用强的松龙0.5 mg/kg/天和局部氯倍他索治疗可显著改善病变。维持治疗:口服甲氨蝶呤15mg /周,同时逐渐减少皮质类固醇,导致皮肤病变完全愈合,无新病变的证据。在瘢痕性脱发的病例中,BPP应被视为鉴别诊断。重要的是要将患者的病史联系起来,询问复发情况,回顾体检结果,以及活检和DIF报告。如果血清学自身抗体鉴定是不可用的,盐分裂试验作为一个有用的和具有成本效益的诊断工具。这是西班牙裔女性患者报告的第三例BPP病例。 所有作者都参与了文章的构思、设计、起草和修改。所有作者都阅读并批准了最终的手稿。所有作者对数据的完整性和准确性负责。根据适用的伦理准则,该项目由医院伦理与研究委员会审查并批准。本文中的所有患者均已书面知情同意参与研究,并同意使用其去身份化、匿名化、汇总的数据和病例详细信息(包括照片)进行发表。作者声明无利益冲突。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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