Cdags syndrome

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Definition
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Acronym for "craniosynostosis and clavicular hypoplasia, delayed closure of the fontanel, cranial defects and deafness, anal anomalies, genitourinary malformations and skin eruptions", a congenital systemic disease whose symptoms manifest themselves in the first months of life, and which has been defined as porokeratosis in some patients.

Occurrence/Epidemiology
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Very rare clinical picture

Etiopathogenesis
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The genetic defect is located in the chromosome region 22q12-q13.

Manifestation
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Infancy

Clinical features
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Clinically dermatologically, therapy-resistant acral accentuated, eczematous changes may occur which are reminiscent of " Acrodermatitis enteropathica".

Therapy
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Symptomatic, anti-eczematous.

Literature
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  1. Kerstan A et al (2011) Unusual dermatitis in CDAGS syndrome: clinical differential diagnosis of acrodermatitis enteropathica and dysmetabolica. Abstract CD 46th DDG meeting FV02/08

Outgoing links (1)

Acrodermatitis enteropathica;

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020