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Bullöse Autoimmunerkrankungen des Kindesalters

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Zusammenfassung

Blasenbildende Autoimmundermatosen sind meist schwer und chronisch verlaufende Erkrankungen der Haut und Schleimhäute, die selten auch bei Kindern auftreten. Zugrunde liegt eine Spaltbildung in der dermoepidermalen Junktionszone oder innerhalb der Epidermis, die meist durch gegen Adhäsionsstrukturen der Haut gerichtete zirkulierende Autoantikörper hervorgerufen wird. Die im Kindesalter häufigste dieser Erkrankungen ist die lineare IgA-Dermatose; Pemphigoid- und Pemphiguserkrankungen, Epidermolysis bullosa acquisita und Dermatitis herpetiformis treten demgegenüber deutlich seltener auf. Häufiger als im Erwachsenalter sind bei Kindern Schleimhäute beteiligt. In den letzten Jahren ist die Identifikation der meisten der zugrunde liegenden Autoantigene gelungen. Dies hat zu einer Verbesserung der Diagnostik geführt und erleichtert Therapieentscheidungen. Zur Behandlung ist in der Regel eine systemische Immunsuppression erforderlich. Angesichts des häufig langjährigen und mitunter komplizierten Verlaufs blasenbildender Autoimmundermatosen ist eine enge Zusammenarbeit zwischen Dermatologen und Pädiatern anzustreben.

Abstract

Autoimmune blistering skin diseases are exceedingly rare in children. In most cases, they are characterized by circulating autoantibodies directed against adhesion structures of the skin which subsequently induce blistering at the dermoepidermal junction or intraepidermally. The most frequent paediatric immunobullous disease is linear IgA dermatosis; all others such as pemphigoid, epidermolysis bullosa acquisita, dermatitis herpetiformis and pemphigus are less common than in adults. In children, mucous membranes are involved more frequently. Recent advances in the identification of the autoantigens have improved diagnostic approach and therapeutic management of blistering diseases. In most cases, treatment requires systemic immunosuppression. With respect to the chronic course of the diseases and potential complications of treatment, cooperation between dermatologists and paediatricians seems advisable.

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Danksagung

Die Autoren danken Dr. Cassian Sitaru und Dr. Christian Rose, Würzburg, Dr. Thomas Wygold, Lübeck, sowie Professor Dr. Lutz Weber, Ulm, für die Bereitstellung von Abbildungsmaterial.

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  1. M. Goebeler

    Present address: Klinik und Poliklinik für Haut- und Geschlechtskrankheiten der Universität Würzburg, , Josef-Schneider-Straße 2, 97080, Würzburg

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  1. Klinik und Poliklinik für Haut- und Geschlechtskrankheiten, Universität Würzburg

    M. Goebeler & D. Zillikens

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Correspondence to M. Goebeler.

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Goebeler, M., Zillikens, D. Bullöse Autoimmunerkrankungen des Kindesalters. Hautarzt 54, 14–24 (2003). https://doi.org/10.1007/s00105-002-0466-1

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