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Hidrotic Ectodermal Dysplasias

  • Conference paper
Pediatric Dermatology

Abstract

Ectodermal dysplasias (EDs) represent a large heterogeneous group of different malformations of cutaneous appendages. The lowest common denominator of these EDs is disorders of at least two ectodermal differentiation products [7, 31]: (1) hair anomaly, (2) dental anomaly, (3) onychodysplasia, or (4) sweat gland anomaly. Neuroectodermal and further malformations may occur in addition. The X-linked anhidrotic EDs, characterized by their main feature of hypohidrosis or anhidrosis, are most widely known. They stand out by their relatively constant clinical pattern. Hidrotic EDs are considerably more variable. Their multiplicity is now far from easy to survey. Fifty to 100 different disorders would easily be classified as hidrotic EDs (HEDs) with a broad application of the term ED. Most of these HED types, however, are extremely rare and many are single cases. In addition, many disorders are wrongly classified as EDs (secondary involvement of ectodermal differentiation products due to different complex disorders, combination of keratinization disorders with malformation of only one ectodermal differentiation product, variable affection of ectodermal differentiation products in inborn and acquired metabolic diseases and malformation syndromes, and phenocopies) though the malformation of ectodermal differentiation products — skin, appendages, and teeth — is secondary to other alterations. According to the above-mentioned definition, malformations of only one ectodermal differentiation product in combination with ichthyoses or palmoplantar keratoses as well as variable hair and nail changes due to inborn errors of metabolism are not EDs.

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Author notes

  1. E. Haneke

    Present address: Hautklinik, Arrenbergerstr. 20, D-5600, Wuppertal, Germany

Authors and Affiliations

  1. Dermatologische Klinik, Universität Erlangen-Nürnberg, Hartmannstr. 14, D-8520, Erlangen, Germany

    E. Haneke

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  1. E. Haneke
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Editors and Affiliations

  1. Afdeling Huidziekten, Sint Radboudziekenhuis, Javastraat 104, NL-6524 MJ, Nijmegen, The Netherlands

    Rudolf Happle

  2. Clinique Dermatologique, 1, Place de l’Hôpital, F-67091, Strasbourg, France

    Edouard Grosshans

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© 1987 Springer-Verlag Berlin Heidelberg

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Haneke, E. (1987). Hidrotic Ectodermal Dysplasias. In: Happle, R., Grosshans, E. (eds) Pediatric Dermatology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-71524-2_5

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  • DOI: https://doi.org/10.1007/978-3-642-71524-2_5

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-540-16991-8

  • Online ISBN: 978-3-642-71524-2

  • eBook Packages: Springer Book Archive

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