Couselo-Rodríguez Carmen, Batalla-Cebey Ana, Álvarez-Álvarez Carlos, Flórez Ángeles
Servicio de Dermatología del Complejo Hospitalario Universitario de Pontevedra, Pontevedra, España.
Grupo de Investigación Dermatología Inflamatoria, Pediátrica y Oncológica (DIPO), Instituto de Investigación Sanitaria Galicia Sur, Servizo Galego de Saude (SERGAS)-Universidad de Vigo (UVIGO).
Arch Argent Pediatr. 2021 Dec;119(6):e636-e638. doi: 10.5546/aap.2021.e636.
Ulerythema ophryogenes is a benign and rare skin disorder commonly presenting in childhood. It is characterized by erythematous and keratotic follicular papules located on the side of the eyebrows, and which over time tends to evolve into scarred alopecia. This entity may appear as an isolated clinical manifestation or associated with several congenital syndromes (18p-, Cornelia de Lange, Noonan, Rubinstein- Taybi, among others). Uleritema ofriógenes como entidad asociada al síndrome 18pen un paciente pediátrico Ulerythema ophryogenes as an entity associated with 18p- syndrome in a pediatric patient We present a 13-year-old male with 18p- syndrome who consults for rough lesions and hair loss in both eyebrows (ulerythema ophryogenes), as well as for hyperkeatosis pilaris in both arms. This triad, known as Zouboulis syndrome, has been rarely reported in the literature. We consider that the recognition of ulerythema ophryogenes is of crucial importance since, in view of its presence, comprehensive anamnesis and physical examination should be performed in search of other alterations that could guide the existence of an underlying genetic disorder.
眉部毛发角化性红斑是一种常见于儿童期的良性罕见皮肤病。其特征为位于眉毛一侧的红斑性和角化性毛囊丘疹,随着时间推移往往会发展为瘢痕性脱发。该病症可能表现为孤立的临床表现,或与多种先天性综合征相关(如18p-综合征、科妮莉亚·德·朗格综合征、努南综合征、鲁宾斯坦-泰比综合征等)。一名患有18p-综合征的儿科患者出现眉部毛发角化性红斑 我们报告一名13岁患有18p-综合征的男性患者,因双侧眉毛出现粗糙皮损和脱发(眉部毛发角化性红斑)以及双侧手臂出现毛发角化病前来就诊。这种三联征,即所谓的祖布利兹综合征,在文献中鲜有报道。我们认为认识眉部毛发角化性红斑至关重要,因为鉴于其存在,应进行全面的病史采集和体格检查,以寻找可能提示潜在遗传疾病存在的其他异常情况。