- Author:
Jong Keun SEO
1
;
Ju Hyun KANG
;
Hyun Jae LEE
;
Deborah LEE
;
Ho Suk SUNG
;
Seon Wook HWANG
Author Information
- Publication Type:Case Report
- Keywords: Adams-Oliver syndrome; Aplasia cutis congenita; Cutis marmorata telangiectatica
- MeSH: Ectodermal Dysplasia; Extremities; Limb Deformities, Congenital; Prognosis; Scalp Dermatoses; Skin; Urogenital System
- From:Annals of Dermatology 2010;22(1):96-98
- CountryRepublic of Korea
- Language:English
- Abstract: 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.