Ann Dermatol.  2010 Feb;22(1):96-98. 10.5021/ad.2010.22.1.96.

A Case of Adams-Oliver Syndrome

• Seo JK
• Kang JH
• Lee HJ
• Lee D
• Sung HS
• Hwang SW

• KMID: 2172050
• DOI: http://doi.org/10.5021/ad.2010.22.1.96

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.

Keyword

Adams-Oliver syndrome; Aplasia cutis congenita; Cutis marmorata telangiectatica

MeSH Terms

Ectodermal Dysplasia
Extremities
Limb Deformities, Congenital
Prognosis
Scalp Dermatoses
Skin
Urogenital System
Ectodermal Dysplasia
Limb Deformities, Congenital
Scalp Dermatoses

Figure

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  Fig. 1 Alopecic scar of aplasia cutis (10×12 cm) at the vertex.

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  Fig. 2 (A) Brachydactyly and cutis marmorata telangiectatica on the dorsa of both feet and (B) the absence of the right 3rd toenail.

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  Fig. 3 Histopathologically, the hairless atrophic patch on the scalp showed rete ridge flattening, collagen bundle thickening, and loss of appendage (H&E, ×40).

Reference

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