The wide spectrum of clinical expression in Adams-Oliver syndrome: A report of two cases

M. Mempel; D. Abeck; I. Lange; K. Strom; A. Caliebe; A. Beham; M. Kautza; W. I. Worret; B. A. Neubauer; J. Ring; H. Schröder; R. Fölster-Holst

doi:10.1046/j.1365-2133.1999.02881.x

The wide spectrum of clinical expression in Adams-Oliver syndrome: A report of two cases

M. Mempel, D. Abeck, I. Lange, K. Strom, A. Caliebe, A. Beham, M. Kautza, W. I. Worret, B. A. Neubauer, J. Ring, H. Schröder, R. Fölster-Holst

TUM Emeriti of Excellence

Christian-Albrechts-University of Kiel

Research output: Contribution to journal › Article › peer-review

45 Scopus citations

Abstract

Two children are described with the combination of aplasia cutis congenita (ACC) and transverse limb defects known as Adams-Oliver syndrome. Whereas in the first child the typical features of ACC, syndactyly and transverse nail dystrophy were only mildly expressed and associated defects of the central nervous system and cardiac malformations were absent, the second child suffered from a very severe expression of the syndrome, with a combination of ACC, syndactyly, cutis marmorata telangiectatica congenita and multiple cardiac and central nervous system malformations which resulted in fatal central respiratory insufficiency.

Original language	English
Pages (from-to)	1157-1160
Number of pages	4
Journal	British Journal of Dermatology
Volume	140
Issue number	6
DOIs	https://doi.org/10.1046/j.1365-2133.1999.02881.x
State	Published - 1999

Keywords

Adams-Oliver syndrome
Aplasia cutis congenita
Cutis marmorata telangiectatica congenita
McKusick 10030
Syndactyly

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10.1046/j.1365-2133.1999.02881.x

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abstract = "Two children are described with the combination of aplasia cutis congenita (ACC) and transverse limb defects known as Adams-Oliver syndrome. Whereas in the first child the typical features of ACC, syndactyly and transverse nail dystrophy were only mildly expressed and associated defects of the central nervous system and cardiac malformations were absent, the second child suffered from a very severe expression of the syndrome, with a combination of ACC, syndactyly, cutis marmorata telangiectatica congenita and multiple cardiac and central nervous system malformations which resulted in fatal central respiratory insufficiency.",

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AU - Mempel, M.

AU - Abeck, D.

AU - Lange, I.

AU - Strom, K.

AU - Caliebe, A.

AU - Beham, A.

AU - Kautza, M.

AU - Worret, W. I.

AU - Neubauer, B. A.

AU - Ring, J.

AU - Schröder, H.

AU - Fölster-Holst, R.

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AB - Two children are described with the combination of aplasia cutis congenita (ACC) and transverse limb defects known as Adams-Oliver syndrome. Whereas in the first child the typical features of ACC, syndactyly and transverse nail dystrophy were only mildly expressed and associated defects of the central nervous system and cardiac malformations were absent, the second child suffered from a very severe expression of the syndrome, with a combination of ACC, syndactyly, cutis marmorata telangiectatica congenita and multiple cardiac and central nervous system malformations which resulted in fatal central respiratory insufficiency.

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KW - Aplasia cutis congenita

KW - Cutis marmorata telangiectatica congenita

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KW - Syndactyly

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The wide spectrum of clinical expression in Adams-Oliver syndrome: A report of two cases

Abstract

Keywords

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