Ocampo-Garza Sonia Sofía, Orizaga-Y-Quiroga Thelma Laura, Olvera-Rodríguez Valeria, Herz-Ruelas Maira Elizabeth, Chavez-Alvarez Sonia, Vañó-Galván Sergio, Ocampo-Candiani Jorge
Universidad Autónoma de Nuevo León, Hospital Universitario "Dr. José Eleuterio González", Dermatology Department, Monterrey, Mexico.
Escuela de Medicina y Ciencias de la Salud, Tecnológico de Monterrey, Monterrey, Mexico.
Skin Appendage Disord. 2021 Apr;7(3):206-211. doi: 10.1159/000512039. Epub 2021 Mar 4.
Frontal fibrosing alopecia (FFA) is an acquired primary lymphocytic cicatricial alopecia characterized by frontotemporal hairline recession, leading to scarring alopecia with a band-like distribution. Prevalence is increasing worldwide, being the most frequent cause of primary scarring alopecia. The natural history of this condition is variable; however, slow progression with spontaneous remission is the most frequent reported outcome. The etiopathogenesis of FFA remains to be elucidated; numerous hypotheses concerning hormonal effects, environmental factors, and genetic predisposition have been proposed. Special interest on genetic basis has emerged since the first familial case was reported. Only a few more familial cases have been published. We report 6 additional cases of female patients with familial FFA (F-FFA) from 3 different families. Sixty-six percent had a family history of autoimmune disease in first-degree relatives; these same patients had a personal history of autoimmune disease. The families described in this cohort study plus the personal and family history of autoimmune disease, as well as the recently described involved genomic loci; reinforced the hypothesis of this disease being genetic. It is important to consider studying this entity since there are scarce data regarding familial cases and this might give us a better insight toward understanding its pathogenesis.
额部纤维性秃发(FFA)是一种获得性原发性淋巴细胞性瘢痕性秃发,其特征为额颞部发际线后移,导致呈带状分布的瘢痕性秃发。在全球范围内,其患病率正在上升,是原发性瘢痕性秃发最常见的病因。这种疾病的自然病程各不相同;然而,最常报道的结果是缓慢进展并伴有自发缓解。FFA的发病机制仍有待阐明;关于激素作用、环境因素和遗传易感性已提出了众多假说。自首例家族性病例报道以来,人们对其遗传基础产生了特别的兴趣。此后仅发表了少数几例家族性病例。我们报告了来自3个不同家族的另外6例家族性FFA(F-FFA)女性患者。66%的患者一级亲属中有自身免疫性疾病家族史;这些患者本人也有自身免疫性疾病病史。本队列研究中描述的家族以及自身免疫性疾病的个人和家族病史,再加上最近描述的相关基因组位点,强化了这种疾病具有遗传性的假说。考虑对这一实体进行研究很重要,因为关于家族性病例的数据很少,这可能会让我们更好地洞察其发病机制。