Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2010 Feb;22(1):96-8.
doi: 10.5021/ad.2010.22.1.96. Epub 2010 Feb 28.

A case of adams-oliver syndrome

Jong-Keun Seo  1 , Ju-Hyun Kang, Hyun-Jae Lee, Deborah Lee, Ho-Suk Sung, Seon-Wook Hwang
Affiliations
Case Reports

A case of adams-oliver syndrome

Jong-Keun Seo et al. Ann Dermatol. 2010 Feb.

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.

Keywords: Adams-Oliver syndrome; Aplasia cutis congenita; Cutis marmorata telangiectatica.

PubMed Disclaimer

Figures

Fig. 1
Fig. 1
Alopecic scar of aplasia cutis (10×12 cm) at the vertex.
Fig. 2
Fig. 2
(A) Brachydactyly and cutis marmorata telangiectatica on the dorsa of both feet and (B) the absence of the right 3rd toenail.
Fig. 3
Fig. 3
Histopathologically, the hairless atrophic patch on the scalp showed rete ridge flattening, collagen bundle thickening, and loss of appendage (H&E, ×40).
See this image and copyright information in PMC

References

    1. Adams FH, Oliver CP. Hereditary deformities in man: due to arrested development. J Hered. 1945;36:3–7.
    1. Mempel M, Abeck D, Lange I, Strom K, Caliebe A, Beham A, et al. The wide spectrum of clinical expression in Adams-Oliver syndrome: a report of two cases. Br J Dermatol. 1999;140:1157–1160. - PubMed
    1. Becker R, Kunze J, Horn D, Gasiorek-Wiens A, Entezami M, Rossi R, et al. Autosomal recessive type of Adams-Oliver syndrome: prenatal diagnosis. Ultrasound Obstet Gynecol. 2002;20:506–510. - PubMed
    1. Narang T, Kanwar AJ, Dogra S. Adams-Oliver syndrome: a sporadic occurrence with minimal disease expression. Pediatr Dermatol. 2008;25:115–116. - PubMed
    1. Dadzie OE, Tyszczuk L, Holder SE, Teixeira F, Charakida A, Scarisbrick J, et al. Adams-Oliver syndrome with widespread CMTC and fatal pulmonary vascular disease. Pediatr Dermatol. 2007;24:651–653. - PubMed

Publication types