Tonolli Vanessa Mello, Stolf Hamilton Ometto, Tonello Cláudio Sampieri, Pires Rafaelle Batistella, Abbade Luciana Patricia Fernandes
"Júlio de Mesquita Filho" Paulista State University, Botucatu, SP, Brasil.
Lauro de Souza Lima Hospital, Bauru, SP, Brazil.
An Bras Dermatol. 2014 Mar-Apr;89(2):363-4. doi: 10.1590/abd1806-4841.20142806.
Hay-Wells syndrome or AEC (Ankyloblepharon, Ectodermal dysplasia and Cleft lip and palate syndrome) is a rare ectodermal disorder. The treatment is aimed to prevent clinical complications. We describe the case of a four-month old male patient with erosions on the scalp, trunk and arms, trachyonychia, deformity of the ears, micropenis, cleft palate, decreased eyebrow and eyelash hairs, in addition to antecedents of surgical correction of ankyloblepharon. The importance of the correct diagnosis is emphasized, besides the investigation of the associated diseases, treatment of complications and genetic counseling of the parents.
海-韦综合征或AEC（睑缘粘连、外胚层发育不良和唇腭裂综合征）是一种罕见的外胚层疾病。治疗旨在预防临床并发症。我们描述了一名4个月大男性患者的病例，该患者头皮、躯干和手臂有糜烂，有粗糙甲，耳部畸形，小阴茎，腭裂，眉毛和睫毛稀少，此外还有睑缘粘连手术矫正史。强调了正确诊断的重要性，以及对相关疾病的调查、并发症的治疗和对父母的遗传咨询。
