Kinder- und Jugendmedizin 2009; 9(01): 14-21
DOI: 10.1055/s-0038-1629010
Schattauer GmbH

Pigmentstörungen im Kindesalter

Pigmentary disorders in childhood
A. Hartmann
1   Hautklinik, Friedrich-Alexander-Universität Erlangen
H. Hamm
2   Klinik für Dermatologie, Allergologie und Venerologie, Julius-Maximilians-Universität Würzburg
› Author Affiliations
Further Information

Publication History

Eingereicht am: 19 May 2008

angenommen am: 02 June 2008

Publication Date:
25 January 2018 (online)


Störungen des Pigmentsystems manifestieren sich häufig bereits im Kindesalter. Während bei Hypomelanosen eine Verminderung des Melanins, z. B. durch defekte Synthese oder Mangel an Melanozyten zugrunde liegt, werden viele Hypermelanosen durch Vermehrung von Melanozyten bzw. verstärkte Speicherung von Melanin hervorgerufen. Die Pathomechanismen sind vielfältig. Unterscheiden lassen sich umschriebene, disseminierte und generalisierte Pigmentstörungen sowie epidermal und dermal gelegene. Sie können erworben sein, wie Vitiligo und postinflammatorische Hypo- und Hyperpigmentierungen, angeboren, wie Naevus depigmentosus und Mongolenfleck, oder durch monogene Erbleiden verursacht sein, wie Piebaldismus und Albinismus. Besondere Bedeutung haben Störungen der Pigmentie-rung im Rahmen von Systemkrankheiten wie neurokutanen Syndromen oder Pigmentmosaiken, da sie Initialsymptom sein und auf assoziierte Manifestationen an anderen Organen hinweisen können. Die vorliegende Arbeit über eine Auswahl angeborener und erworbener Pigmentstörungen im Säuglings- und Kindes-alter stellt besonders jene heraus, die häufig vorkommen und die im Rahmen komplexer Syndrome diagnostisch bedeutsam sind.


Pigmentary disorders often become evident already in childhood. Hypomelanoses are based on a decrease in melanin content, for example due to defective melanin synthesis or lack of melanocytes, whereas many hypermelanoses are caused by an increase in melanocytes or storage of melanin. The pathomechanisms are manifold. Pigmentary abnormalities may follow a circumscribed, disseminated, or generalized pattern and the pathology may be located in the epidermis or dermis. They may be acquired, as vitiligo and postinflammatory hypo- and hyperpigmentations, congenital, as naevus depigmentosus and Mongolian spot, or caused by monogenic traits, like piebaldism and albinism. Pigmentary lesions are of particular importance in the context of systemic diseases, e. g. neurocutaneous syndromes and pigmentary mosaicism, as they may be the initial sign and indicative for associated abnormalities of other organs. The present work on a selection of congenital or acquired pigmentary disorders in infancy and childhood gives special emphasis to frequent conditions and to findings which are of diagnostic significance in the scope of complex syndromes.

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