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Kutaner Lupus erythematodes

Teil 2: Diagnostik und Therapie

Cutaneous lupus erythematosus

Part 2: Diagnostics and therapy

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Zusammenfassung

Der kutane Lupus erythematodes (CLE) bedarf einer gezielten Diagnostik, um die einzelnen Subtypen von verschiedenen Differenzialdiagnosen abzugrenzen und systemische Organmanifestationen auszuschließen. Zusätzlich zu einer ausführlichen Anamnese und einer klinischen Beurteilung der Hautveränderungen sind histopathologische und immunfluoreszenzmikroskopische Untersuchungen einer Biopsie sowie ein Laborscreening zu empfehlen. Eine Photoprovokationstestung kann bei Patienten mit CLE zur Einschätzung der Photosensitivität und zur Bestätigung der Diagnose durchgeführt werden. Zur Beurteilung der Aktivität der kutanen Manifestationen wurde kürzlich ein Punktwertsystem entwickelt und validiert, das sowohl die anatomische Region als auch die Morphe der einzelnen Hautläsionen berücksichtigt. Auch heute noch sind die Antimalariamittel bei allen Subtypen des CLE die Therapie der 1. Wahl. Fortschritte in der Biotechnologie führten zwar zur Entwicklung von zahlreichen neuen Substanzen für die Therapie von Autoimmunerkrankungen, die aber bisher für den CLE nicht in kontrollierten Studien evaluiert worden sind. Weiterhin besteht Bedarf für eine spezifische immunologische Behandlung, insbesondere für Patienten, die nicht auf eine standardisierte Therapie ansprechen. Der 2. Teil dieses Weiterbildungsbeitrags befasst sich mit der differenzialdiagnostischen Abgrenzung sowie der gezielten Diagnostik und Therapie des CLE.

Abstract

The diagnosis of cutaneous lupus erythematosus (CLE) requires a specific diagnostic approach to identify subtypes, to address differential diagnostic considerations,

and to rule out systemic organ involvement. In addition to a detailed patient’s history and clinical evaluation of the skin, histopathologic and immunofluorescent examination of a skin biopsy as well as laboratory screening are recommended. Photoprovocation tests can be performed to confirm the diagnosis of CLE and to assess photosensitivity in these patients. Recently, a scoring system for the activity of the cutaneous manifestations in CLE has been developed and validated which involves anatomical areas and morphologic signs of the skin lesions. In all subtypes of CLE, antimalarials are still the treatment of choice. Advances in biotechnology have led to the development of several novel agents for the treatment of autoimmune diseases; however, controlled trials have not been performed in patients with CLE. Furthermore, there is need for specific immunointervention, especially for patients who fail to respond to standard therapies. The second part of this review will enable the reader to differentiate CLE from other diseases and to suggest specific diagnostic procedures and treatment approaches.

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Abbreviations

ACLE:

Akut kutaner Lupus erythematodes

ACR:

American College of Rheumatology

ANA:

Antinukleäre Antikörper

BILAG:

British Isles Lupus Assessment Group

CCLE:

Chronisch kutaner Lupus erythematodes

CHLE:

Chilblain Lupus erythematodes

CLASI:

Cutaneous Lupus Erythematosus Disease Area and Severity Index

CLE:

Kutaner Lupus erythematodes

DIF:

Direkte Immunfluoreszenz

DLE:

Diskoider Lupus erythematodes

dsDNS Antikörper:

Doppelstrang-DNS Antikörper

ECLAM:

European Consensus Lupus Activity Measurement

EEM:

Erythema exsudativum multiforme

ICLE:

Intermittierender kutaner Lupus erythematodes

LEHV:

Lupus erythematodes hypertrophicus/verrucosus

LEP:

Lupus erythematodes profundus

LET:

Lupus erythematodes tumidus

NLE:

Neonataler Lupus erythematodes

PLD:

Polymorphe Lichtdermatose

SLEDAI:

Systemic Lupus Erythematosus Disease Activity Index

SCLE:

Subakut kutaner Lupus erythematodes

SLE:

Systemischer Lupus erythematodes

TEN:

Toxische epidermale Nekrolyse

UV-Licht:

Ultraviolettes Licht

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Danksagung

Die Abbildungen wurden freundlicherweise vom Fotolabor der Hautklinik des Universitätsklinikums Münster (mit Dank an Herrn P. Wissel, Frau J. Bückmann und Herrn Prof. Dr. T.A. Luger) und der Hautklinik der Heinrich-Heine-Universität Düsseldorf (mit Dank an Herrn W. Neuse und Herrn Prof. Dr. Dr. h.c. T. Ruzicka) überlassen.

Diese Arbeit wurde durch ein Heisenberg-Stipendium von der Deutschen Forschungsgemeinschaft (DFG) an A.K. (KU 1559/1-1) unterstützt.

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  1. Hautklinik, Heinrich-Heine-Universität Düsseldorf, Moorenstraße 5, 40225 , Düsseldorf, Germany

    A. Kuhn & K. Gensch

  2. Hautklinik des Universitätsklinikums Münster, Münster, Germany

    S. Ständer & G. Bonsmann

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  1. A. Kuhn
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Kuhn, A., Gensch, K., Ständer, S. et al. Kutaner Lupus erythematodes. Hautarzt 57, 345–360 (2006). https://doi.org/10.1007/s00105-006-1138-3

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