Der Lichen planus des Ösophagus – Eine unterschätzte Erkrankung

Zusammenfassung

Eine Beteiligung des Ösophagus bei der Hauterkrankung Lichen planus wurde erstmals 1982 beschrieben und fast 30 Jahre lang als eine Rarität angesehen. Untersuchungen der letzten 10 Jahre aber zeigen, dass diese Erkrankung weniger selten ist als angenommen. Es ist sogar anzunehmen, dass der ösophageale Lichen planus (Esophageal Lichen planus, ELP) häufiger ist als die Eosinophile Ösophagitis (EoE). Die Ösophagusbeteiligung betrifft meist Frauen im mittleren Alter. Das Hauptsymptom ist eine Dysphagie. Endoskopisch erkennt man in der Speiseröhre eine charakteristische Schleimhautablösung, eine Trachealisierung, und gelegentlich Hyperkeratosen und bei langem Bestehen auch Stenosen. Wegweisend ist die Histologie mit einer subepithelialen Ablösung sowie einem bandförmigen Infiltrat aus T-Lymphozyten, dem Nachweis von apoptotischen Keratinozyten (Civatte Bodies) und Dyskeratosen. Die direkte Immunfluoreszenz zeigt Fibrinogen-Ablagerungen entlang der Basalmembran. Eine etablierte Therapie gibt es bisher nicht. Die Behandlung mit topischen Steroiden ist in 2/3 der Fälle wirksam. Eine Therapie wie beim klassischen Lichen planus scheint unwirksam zu sein. Bei symptomatischen Stenosen kann eine Dilatation indiziert sein. Der ELP reiht sich in die Gruppe der „neuen“ immunologisch vermittelten Erkrankungen des Ösophagus ein.

Abstract

An involvement of the esophagus in patients with lichen planus was described for the first time in 1982. Ever since, it has been seen as a rarity. However, studies over the last 10 years have shown a higher prevalence than expected. It may even be supposed that esophageal lichen planus (ELP) is more common than eosinophilic esophagitis. ELP mostly affects middleaged women. The principal symptom is dysphagia. Endoscopically, ELP is characterized by denudation and tearing of the mucosa, trachealization and hyperkeratosis and esophageal stenosis may occur in patients with long courses of the disease. Histologic findings including mucosal detachment, T-lymphocytic infiltrate, intraepithelial apoptosis (civatte bodies) and dyskeratosis are crucial. Direct immunofluorescence shows fibrinogen deposits along the basement membrane zone. So far, there is no well-established therapy but a treatment with topic steroids is effective in 2/3 of the patients. Common therapy of lichen planus of the skin seems to be ineffective for treatment of ELP. Symptomatic esophageal stenosis should be endoscopically dilated. ELP joins the group of „new“ immunologic diseases of the esophagus.

Schlüsselwörter

Lichen planus - Esophagitis - Eosinophilic esophagitis - Dysphagia - Budesonide - Autoimmune disorder - T-lymphocytes

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