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Kutaner Lupus erythematodes

Teil 1: Klinik und Klassifikation

Cutaneous lupus erythematosus

Part 1: Clinical manifestations and classification

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Zusammenfassung

Der kutane Lupus erythematodes (CLE) ist eine heterogene Erkrankung mit einem breiten Spektrum von kutanen Manifestationen, die es nicht erlauben, ein einheitliches Krankheitsbild zu definieren. Im Jahr 2004 wurde die Klassifikation des CLE aktualisiert und umfasst heute den akuten CLE (ACLE), subakuten CLE (SCLE), chronischen CLE (CCLE) und intermittierenden CLE (ICLE). Zusätzliche Formen, die in der Literatur vereinzelt beschrieben worden sind, werden nicht mehr als eigene Entität aufgeführt, sondern den klassischen Formen zugeteilt. Die Diagnose der verschiedenen Subtypen des CLE wird mit Hilfe genetischer, klinischer, histopathologischer und immunserologischer Befunde bestätigt und bedarf einer gezielten Analyse der einzelnen Kriterien. In den letzten Jahren war die Ätiologie und Pathogenese des CLE Gegenstand intensiver Forschung, und zahlreiche Untersuchungen konnten zeigen, dass exogene Faktoren, insbesondere ultraviolettes Licht und Medikamente, die Entstehung dieser Erkrankung begünstigen Manifestationen. Der 1. Teil dieses Weiterbildungsbeitrags soll dem Leser ermöglichen, die klinischen Subtypen des CLE zu erkennen und mit Hilfe charakteristischer Kriterien von Differenzialdiagnosen abzugrenzen.

Abstract

Cutaneous lupus erythematosus (CLE) is a heterogenous disorder with a wide range of skin manifestations. Therefore, it has been difficult to develop a unifying concept for classifying CLE from the dermatologic perspective in the past. In 2004, the classification system was updated and includes now acute CLE (ACLE), subacute CLE (SCLE), chronic CLE (CCLE), and intermittent CLE (ICLE). Additional rarely described variants are not listed as separate entities but are included in the classical forms. Diagnosis of the different subtypes of CLE is made by considering genetic, clinical, histopathologic, and immunoserologic findings, with a systematic analysis of individual criteria. In the past years, the etiology and pathogenesis of CLE has been subject of intensive research and it has been shown by several groups that exogenous factors, such as ultraviolet light and drugs, can induce CLE. The first part of this review will enable the reader to identify the various clinical manifestations of CLE and to employ characteristic criteria to assess differential diagnostic considerations.

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Abbreviations

ACLE:

Akut kutaner Lupus erythematodes

ACR:

American College of Rheumatology

ANA:

Antinukleäre Antikörper

CCLE:

Chronisch kutaner Lupus erythematodes

CHLE:

Chilblain Lupus erythematodes

CLE:

Kutaner Lupus erythematodes

DIF:

Direkte Immunfluoreszenz

DLE:

Diskoider Lupus erythematodes

dsDNS-Antikörper:

Doppelstrang-DNS-Antikörper

EEM:

Erythema exsudativum multiforme

ENA:

Extrahierbare nukleäre Antikörper

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

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 der Heinrich-Heine-Universität Düsseldorf

    A. Kuhn & K. Gensch

  2. Hautklinik des Universitätsklinikums Münster

    S. Ständer & G. Bonsmann

  3. Hautklinik, Heinrich-Heine-Universität Düsseldorf, Moorenstraße 5, 40225 , Düsseldorf

    A. Kuhn

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  1. A. Kuhn
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Kuhn, A., Gensch, K., Ständer, S. et al. Kutaner Lupus erythematodes. Hautarzt 57, 251–268 (2006). https://doi.org/10.1007/s00105-006-1094-y

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