Marukian Nareh V, Levinsohn Jonathan L, Craiglow Brittany G, Milstone Leonard M, Choate Keith A
Department of Dermatology, School of Medicine, Yale University, New Haven, Connecticut.
Department of Pediatrics, School of Medicine, Yale University, New Haven, Connecticut.
Pediatr Dermatol. 2017 Mar;34(2):160-162. doi: 10.1111/pde.13057. Epub 2016 Dec 23.
Costello syndrome (CS) is a multisystem congenital disorder characterized by coarse facial features, cardiac defects, intellectual disability, and predisposition to malignancies. Dermatologic findings can include cutaneous papillomas, skin redundancy, acanthosis nigricans, and keratosis pilaris. Palmoplantar keratoderma (PPK) is present in approximately 76% of patients with CS, with disabling functional consequences in severe cases. We report a case of CS with severe PPK that improved dramatically with systemic administration of acitretin 0.3 mg/kg/day.
科斯特洛综合征（CS）是一种多系统先天性疾病，其特征为面部粗糙、心脏缺陷、智力残疾以及易患恶性肿瘤。皮肤表现可包括皮肤乳头状瘤、皮肤赘生、黑棘皮病和毛发角化病。掌跖角化病（PPK）在约76%的CS患者中存在，严重病例会导致功能障碍。我们报告一例患有严重PPK的CS患者，通过每日系统性给予0.3 mg/kg阿维A治疗后病情显著改善。
