Seo Jong-Keun, Kang Ju-Hyun, Lee Hyun-Jae, Lee Deborah, Sung Ho-Suk, Hwang Seon-Wook
Department of Dermatology, Busan Paik Hospital, College of Medicine, Inje University, Busan, Korea.
Ann Dermatol. 2010 Feb;22(1):96-8. doi: 10.5021/ad.2010.22.1.96. Epub 2010 Feb 28.
Adams-Oliver syndrome (AOS) is a congenital condition characterized by aplasia cutis congenita, transverse limb defects, and cutis marmorata telangiectatica. AOS can also be associated with extensive lethal anomalies of internal organs, including the central nervous, cardiopulmonary, gastrourointestinal, and genitourinary systems. Generally, the more severe these interrelated anomalies are, the poorer the prognosis becomes. In the relevant literature on this topic, it is somewhat unclear as to whether the prognosis of AOS without lethal anomalies alters the lifespan. We report a case of AOS with typical skin defects only, and no internal organ anomalies.
亚当斯-奥利弗综合征（AOS）是一种先天性疾病，其特征为先天性皮肤发育不全、肢体横断性缺损和毛细血管扩张性大理石样皮肤。AOS还可能与包括中枢神经、心肺、胃肠和泌尿生殖系统在内的广泛致命性内脏异常相关。一般来说，这些相互关联的异常越严重，预后就越差。在关于该主题的相关文献中，对于无致命性异常的AOS预后是否会影响寿命尚不太明确。我们报告一例仅具有典型皮肤缺损且无内脏异常的AOS病例。
