知道这种疤痕性脱发三联征吗?-读书报告

潘卫利大夫 2019-11-07 14:50:10



Graham-Little syndrome

Graham-Little综合征


Clinical features

临床特征


The clinical triad of scarring alopecia of the scalp, noncicatricial alopecia of pubic and axillary hair, and the development of keratosis pilaris on Multiple hairy areas was originally described in 1914 by Piccardi and in 1915 by Graham-Little in two patients, one of them studied by Lassueur (Lassueur-Graham-Little-Piccardi syndrome).1–3 The disease is more frequent in middle-aged women and the scarring alopecia frequently precedes the other components of the syndrome by months to years. Graham-Little综合征是头皮瘢痕性脱发的临床三联征,包括阴毛、腋毛的非瘢痕性脱发以及多毛部位的毛发角化病。最初于1914年被Piccardi描述,随后1915Graham-Little报道了2例患者,其中一例患者由Lassueur研究发现(Lassueur-Graham-Little-Piccardi综合征)。此病易发生于中年女性,并且疤痕性脱发常比其他症状早几个月甚至几年出现。


Graham-Little syndrome has been associated with classic lichen planus, frontal fibrosing alopecia, lichen ruber ulcerosus, complete androgen insensitivity syndrome (testicular feminization syndrome), hepatitis-B vaccination, and HLA-DR1 type in a mother and her daughter.3,6–11 Graham-Little综合征可与经典的扁平苔癣、前额部纤维性脱发、红色溃疡性苔藓、完全性雄激素不敏感综合征(睾丸雌化综合征)、乙肝疫苗、母亲和其女儿的HLA-DR1型相关。


Pathogenesis and histological features

发病机制和组织学特征

Graham-Little syndrome has usually been considered a variant of lichen planopilaris. However the clinical features differ from ordinary lichen planopilaris and the microscopic appearance is variable, including that of a lichenoid tissue reaction and follicular hyperkeratosis. Graham-Little综合征常被认为是扁平毛发苔癣的一种亚型。但是其临床特点与普通的扁平毛发苔癣不同;两者显微镜下表现包括苔藓样组织反应和毛囊角化亦不同。


Differential diagnosis

鉴别诊断


The most important differential diagnosis is keratosis follicularis spinulosa decalvans. However, the clinical picture of the latter is distinctly different. Keratosis follicularis is frequently familial, starting in infancy and affects atopic individuals. It is characterized by patchy alopecia of the scalp, eyebrows, and eyelashes. The clinical course is unpredictable: some cases regress during puberty and others progress with variable intensity.

最主要是要与棘状秃发性毛发角化症相鉴别。但是棘状秃发性毛发角化症的临床表现有明显的不同。毛囊角化病的表现与本综合征常类似,婴儿期发病并且影响特应性的个体,特征性的表现为头皮、眉毛、睫毛散在片状脱发。其临床病程转归不可预测:部分病例在青春期后缓解,亦可出现不同程度的进展。

浙江省人民医院皮肤科 樊一斌大夫译文)

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