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摘要

颞三角形脱发(TTA)或布劳尔痣，是一种局限的，无疤痕形式的脱发出现在颞区。它也被称为少毛痣。文献报道约55例。病变通常为单侧，很少发生双侧。我们报告一例七岁男童双侧TTA。

简介

颞三角形脱发或布劳尔痣(TTA)是位于额颞区的一种边界清晰的三角形或lancet状的非瘢痕性毛发减少区域[1-4]。病变是永久性的，无症状。它最早是由Sabouraud在1905年描述的[3,6]。1926年，它被Brauer重新定义为Brauer综合征的一部分(局灶性面部真皮发育不良-1型=遗传性对称性太阳穴再生障碍性痣，双颞部先天性皮肤再生障碍性痣)，其特征是颞部皮肤凹陷，类似于“钳痕”[7]。

情况下

一个7岁的男孩提出了局部区域的毛发减少在两个颞顶叶区域。由于他出生时头皮无毛，所以这些损伤没有被注意到。患者的父母第一次注意到脱发病变是在3岁的时候。自那时起，无毛区域的外观没有变化。所有家庭成员都没有类似的脱发。该患者过去曾被不同的全科医生评估为斑秃，并曾接受局部和病灶内类固醇治疗，但没有任何改善。没有宫内压史，也没有产科或后天性创伤史。秃发部位无皮肤缺损。在皮肤科检查中，在两个颞顶叶区域均有较少且细小的毛状三角形秃斑(图1a、1b和1c)。右片(7x9x13厘米)比左片(5x7x9厘米)大。 Dermatoscopic examination showed mostly vellus hairs and a small number of terminal hairs at the alopecic patches (Figure 1d). Between the triangular patches and frontal areas, there were small hair fringes. Any scaling, erythema, scarring or induration were not seen at alopecic areas. Exclamation mark hairs were not seen. Hair pull test was negative at the both area. The dermatological examinations of the nails, mucosa, and the remaining physical examination were normal. Routine laboratory tests were normal. Trichogram or scalp biopsy could not be done because the parents did not give a consent. All of these findings suggested the diagnosis of“双侧先天性颞三角脱发”。

图1所示。典型的三角型稀疏斑的临床观察。a,b)右侧脱发贴片(距离和闭合视图)c)左侧脱发贴片d)右侧脱发贴片的皮肤镜视图。

讨论

TTA通常发生在额颞区，只影响一侧头皮[5]，通常在两岁后出现，这时被末梢毛发取代[1,3,4]。尽管病变的频率尚不清楚，但一些作者报道其频率为0.11%。这似乎对两性都有影响。以前报告的病例主要是在白人中，尽管也有亚裔和非裔美国人[5]病例。TTA的病因目前还不清楚。80%的病例为单侧[1,4,8]，通常为零星出现。然而，它可能在家庭中作为一个突出的特征出现[3,8]。此外，有人认为TTA可能反映了马赛克风格。这种现象通常作为孤立的异常发生，但也可能是多系统出生异常的一部分，如小脑-三叉神经-真皮发育不良(Gomez-Lopez-Hernandez (GLH)综合征)或2型色素血管瘤病[4,6]。另一方面，这种情况可能是常染色体显性遗传[9]综合征的一部分。 Rarely, the lesion may involve large temporoparietal area which can be a characteristic component of GLH syndrome (craniosynostosis, midfacial hypoplasia, trigeminal anesthesia, cerebellar dysplasia and mental deficiency) [5]. Our patient’s lesions were sporadic and we did not detect any familial inheritance. Also, the lesions of the patient were not accompanied by any neurovascular or pigmentary disorders, and they were not parts of the Brauer’s or GLH syndromes. Although rare reports of lesions in the occipital region have been reported, TTA usually affects the frontal and parietotemporal area. Clinically, it is characterized by finer hair in a triangular area with blunt angles and the base of the triangle lying towards the hairline. Sometimes, the shape of the lesion is not exactly resemble a triangle, and can be ovoid or round [5]. Anterior margin of the lesions are sometimes separated from the lesion by a small fringe of normal hair [8], as in our patient. In the affected area, mostly vellus hairs, and occasionally a few terminal hairs are seen. The skin is normal [4,6,8]. Exclamation mark hairs are absent and hair pull test is negative [5]. Histopathologically, hair follicles are markedly miniaturized or absent [6,8,9]. We were unable to histopathological examination of the lesions because the parents did not allow for biopsy. However, the diagnosis of TTA is made based on clinical features [4,6]. A histopathology study is not usually necessary (munios). It should be differentiated primarily from Alopecia Areata (AA), and other patterned hairless lesions such as trichotillomania, tinea capitis, androgenetic alopecia and aplasia cutis [6]. Typical history, precocity, triangular shape, stability over time, poor response to topical steroids and lack of exclamation mark hairs help to distinguish it from AA [4,6]. The lesions of our patient had been noticed at the third year of age when the vellus replaced by terminal hair. He had the typical clinical and dermatoscopic features of TTA ( persistence of the lesions since third age, stable course, typical triangular shapes of the lesions, fronto-temporal locations, presence of the small terminal hair fringe on the border of the frontal regions, absence of the exclamation mark hairs, the normal appearance of the epidermis in affected areas and presence of the mostly vellus hairs on the lesion). There is no specific treatment of this condition and it is not usually necessary [5]. However, the patient can benefit from complete excision of the affected area [6] or hair graft transplantation in the future [3,4,6,10]. Our case was presented for the raise awareness to the rare and unique lesion that very closely mimics alopcia areata. If physicians are aware of the condition, they can explain it to the parents, and patients can be protected from false diagnosis and improper treatments such as corticosteroids.

参考文献

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9. 鲁杰瑞，李志刚，李志刚，等。(2000)一对母女的颞三角形脱发与智力障碍和癫痫的关系。拱北京医学136: 426 - 427。［Crossref］
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