Manel Martinez-Molina, Elena Carmona-Rocha, Javier Gil-Lianes, Delia Yubero, Dídac Casas-Alba, Eulàlia Baselga, Marta Ivars
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引用次数: 0
摘要
口腔-面部-数字综合征 1 型(OFD1)是一种 X 连锁显性发育障碍,由 OFD1 基因突变引起。该病以面部、口腔和数字畸形为特征,但表现各异。皮肤表现很常见(占患者的 20%-30%),特点是粟粒疹和斑片状脱发。三镜检查结果(断发、黑点、丘疹)可能与头癣相似,但此类结果尚未得到很好的描述。临床上,由于三镜检查结果、对长期抗真菌治疗无反应以及中线异常的存在,可高度怀疑外胚层发育不良样综合征,并可通过基因检测确诊。
Trichoscopic findings in neonatal alopecia in oro-facial-digital syndrome type 1.
Oral-facial-digital syndrome type 1 (OFD1) is an X-linked dominant development disorder due to mutations in the OFD1 gene. It is characterized by facial, oral, and digital malformations, although expression is variable. Skin manifestations are frequent (20%-30% of patients) and characterized by evanescent milia and patchy alopecia. Trichoscopic findings (broken hairs, black dots, pili torti) can resemble tinea capitis, although such findings have not been well characterized. High clinical suspicion of ectodermal dysplasia-like syndromes due to trichoscopy findings, absence of response to long-term antifungal therapy, and the presence of midline anomalies can raise suspicion for OFD1, which can be confirmed by genetic testing and enable diagnosis.
期刊介绍:
Pediatric Dermatology answers the need for new ideas and strategies for today''s pediatrician or dermatologist. As a teaching vehicle, the Journal is still unsurpassed and it will continue to present the latest on topics such as hemangiomas, atopic dermatitis, rare and unusual presentations of childhood diseases, neonatal medicine, and therapeutic advances. As important progress is made in any area involving infants and children, Pediatric Dermatology is there to publish the findings.