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Schwere Hautreaktionen: klinisches Bild, Epidemiologie, Ätiologie, Pathogenese und Therapie

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Zusammenfassung

Hintergrund

Schwere Hautreaktionen, meist infolge von Medikamenteneinnahme, sind sehr selten und können mit einer hohen Letalität einhergehen. Eine geeignete Therapie, um die Letalität zu senken, ist erstrebenswert.

Methoden

Die aktuellen Veröffentlichungen zu dieser Thematik wurden gesichtet und bewertet.

Ergebnisse

Bei den selbstlimitierenden Erkrankungen akute generalisierte exanthematische Pustulose (AGEP) und generalisiertes bullöses fixes Arzneiexanthem (GBFDE) gibt es keine klare Indikation für eine systemische immunmodulierende Therapie, der Goldstandard ist die supportive Therapie. Bei „drug reaction with eosinophilia and systemic symptoms“ (DRESS) ist die Situation weniger eindeutig, doch werden systemische Glukokortikosteroide vor allem bei ausgeprägter Organbeteiligung empfohlen. Diese geht mit Komplikationen und häufig auch einer Virusreaktivierung einher, welche die Abheilung verzögern kann. Die Datenlage bei Stevens-Johnson-Syndrom (SJS)/Toxisch epidermaler Nekrolyse (TEN) ist kontrovers für verschiedene immunmodulierende Therapien. Aktuelle Publikationen präferieren eine Steroid-Pulstherapie, den Tumornekrosefaktor(TNF)-α-Inhibitor Etanercept sowie den Calcineurin-Inhibitor Cyclosporin A, wobei letztgenannter den vielversprechendsten Ansatz darstellt.

Schlussfolgerung

Die Seltenheit und Unvorhersehbarkeit der Reaktionen machen eine randomisierte doppelblinde Therapiestudie extrem schwierig. Mithilfe von Metaanalysen kann eine Tendenz in der Anwendung von systemischen Therapieoptionen herausgearbeitet werden. Für alle Krankheitsbilder stellt die supportive Therapie immer noch die wichtigste Behandlungsstrategie dar.

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Abbreviations

AGEP:

Akute generalisierte exanthematische Pustulos

ANA:

Antinukleärer Antikörper

CMV:

Cytomegalievirus

DIHS:

Drug-induced hypersensitivity syndrome

DRESS:

Drug reaction with eosinophilia and systemic symptoms

EBV:

Epstein-Barr-Virus

EEM:

Erythema exsudativum multiforme

EEMM:

Erythema exsudativum multiforme majus

GBFDE:

Generalisiertes bullöses fixes Arzneiexanthem

HHV-6:

Humanes Herpesvirus Typ 6

Ig:

Immunglobulin

IL:

Interleukin

IVIG:

Intravenöse Immunglobuline

KG:

Körpergewicht

KI:

Konfidenzintervall

KOF:

Körperoberfläche

NSAR:

Nicht steroidale Antirheumatika

OR:

Odds Ratio

PCR:

Polymerase chain reaction

SCORTEN:

Severity-of-illness score for TEN

SJS:

Stevens-Johnson-Syndrom

SSSS:

Staphylococcal scalded skin syndrome

Teffs:

Effektor-T-Zellen

TEN:

Toxisch epidermale Nekrolyse

Th:

T-Helferzelle

TNF:

Tumornekrosefaktor

Treg:

Regulatorische T-Zelle

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Authors and Affiliations

  1. Dokumentationszentrum schwerer Hautreaktionen, Klinik für Dermatologie und Venerologie, Universitätsklinikum Freiburg, Freiburg, Deutschland

    Maren Paulmann &  Maja Mockenhaupt

  2. Dokumentationszentrum schwerer Hautreaktionen, Klinik für Dermatologie und Venerologie, Medizinische Fakultät und Universitätsklinikum Freiburg, Hauptstraße 7, 79104, Freiburg, Deutschland

    Maja Mockenhaupt

Authors
  1. Maren Paulmann
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  2. Maja Mockenhaupt
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Correspondence to Maja Mockenhaupt.

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Interessenkonflikt

Die Autorinnen geben an, dass kein Interessenkonflikt vorliegt.

Der Verlag erklärt, dass die inhaltliche Qualität des Beitrags von zwei unabhängigen Gutachtern geprüft wurde. Werbung in dieser Zeitschriftenausgabe hat keinen Bezug zur CME-Fortbildung. Der Verlag garantiert, dass die CME-Fragen frei sind von werblichen Aussagen und keinerlei Produktempfehlungen enthalten. Dies gilt insbesondere für Präparate, die zur Therapie des dargestellten Krankheitsbildes geeignet sind.

Zitierweise

Paulmann M, Mockenhaupt M. Severe skin reactions: clinical picture, epidemiology, etiology, pathogenesis, and treatment. Allergo J Int 2019;28:311—26 https://doi.org/10.1007/s40629-019-00111-8

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Paulmann, M., Mockenhaupt, M. Schwere Hautreaktionen: klinisches Bild, Epidemiologie, Ätiologie, Pathogenese und Therapie . Allergo J 28, 16–34 (2019). https://doi.org/10.1007/s15007-019-1973-z

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  • DOI: https://doi.org/10.1007/s15007-019-1973-z

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