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Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS)

Eine Übersicht

Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS)

A Review

Der Hautarzt volume 64, pages 611–624 (2013)Cite this article

Zusammenfassung

Das DRESS („Drug Reaction with Eosinophilia and Systemic Symptoms“; syn.: Hypersensitivitätssyndrom) ist eine schwere, potenziell lebensbedrohliche Arzneimittelreaktion. Die Mortalität liegt bei 10 %; die Inzidenz ist weltweit zunehmend. Die häufigsten Auslöser sind aromatische Antikonvulsiva (Carbamazepin, Lamotrigin, Phenobarbital) und zunehmend moderne antiretrovirale Medikamente. Die Pathogenese ist nicht vollends geklärt, wahrscheinlich multifaktoriell mit Zusammentreffen von Immunreaktion, ethnischer Prädisposition, genetisch determiniertem Enzymmangel und Reaktivierung von Herpesviren (HHV-6, HHV-7, Epstein-Barr-Virus, Zytomegalievirus). Infolge Haut- und Multiorganbeteiligung ist das klinische Bild variabel, sodass kein eindeutiger klinischer, histologischer oder laborchemischer Phänotyp charakterisiert werden kann. Dies wird durch eine lange Latenz zwischen Beginn der Medikamenteneinnahme und Beginn des DRESS bzw. sukzessives Auftreten von Haut- und Organbeteiligung erschwert. Auch wenn oder gerade weil das DRESS eine diagnostische Herausforderung darstellt, ist eine genaue Kenntnis des Krankheitsbildes wichtig, um frühzeitig therapeutische Maßnahmen einleiten zu können.

Abstract

DRESS (Drug Reaction with Eosinophilia and Systemic Symptoms), also known as hypersensitivity syndrome (HSS), is a severe and potentially life-threatening drug reaction. The worldwide rate of mortality is about 10 %, its incidence is increasing. Drugs that most frequently induce DRESS are aromatic anticonvulsive drugs (carbamazepine, lamotrigine, phenobarbital), and more recently new retroviral therapies. The pathogenesis of DRESS is not yet fully understood, but is certainly multifactorial involving a combination of immune reactions, ethnic predisposition, genetically determined enzyme deficiencies and reactivation of herpes viruses (HHV-6, HHV-7, EBV, CMV). Because of the involvement of the skin and internal organs, the clinical picture can be very variable. No specific clinical, histologic or laboratory parameter is available, so the diagnosis has to rely on the clinical appearance. The long latency period between start of drug intake and the initial manifestation of DRESS and the successive onset of skin and organ involvement complicates the early diagnosis. Although or even because DRESS represents a diagnostic challenge, detailed knowledge about this disease is of utmost importance to enable early therapeutic actions.

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Interessenkonflikt

S. Ständer, D. Metze, T. Luger und T. Schwarz geben an, dass kein Interessenkonflikt besteht. Dieser Beitrag beinhaltet keine Studien an Menschen oder Tieren.

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Affiliations

  1. Kompetenzzentrum Chronischer Pruritus (KCP), Klinik für Hautkrankheiten, Universitätsklinikum Münster, Von-Esmarch-Str. 58, 48149, Münster, Deutschland

    S. Ständer, D. Metze & T. Luger

  2. Klinik für Hautkrankheiten, Universitätsklinikum Schleswig-Holstein, Campus Kiel, Kiel, Deutschland

    T. Schwarz

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  1. S. Ständer
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  2. D. Metze
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Correspondence to S. Ständer.

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Ständer, S., Metze, D., Luger, T. et al. Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS). Hautarzt 64, 611–624 (2013). https://doi.org/10.1007/s00105-013-2615-0

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Schlüsselwörter

  • Typ-IV-Allergie
  • HLA B*5701
  • Hypersensitivitätssyndrom
  • Toxisch epidermale Nekrolyse
  • Arzneimittelreaktion

Keywords

  • Typ IV allergy
  • HLA B*5701
  • Hypersensitivity syndrome
  • Toxic epidermal necrolysis
  • Drug reaction