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Lichen sclerosus vulvae

Die besondere Bedeutung der klinischen und histopathologischen Früherkennung

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Zusammenfassung

Der vulväre Lichen sclerosus (LS) ist eine nicht heilbare und progrediente Hauterkrankung unklarer Ätiologie. Er beginnt schleichend, verläuft unbehandelt bis zur destruktiven Atrophie und ist mit einem erhöhten Plattenepithelkarzinomrisiko assoziiert. Die klassischen Symptome sind Pruritus und Schmerzen, die Frühsymptome jedoch uncharakteristisch; daher werden unklare vulväre Beschwerden zunehmend bioptisch abgeklärt. Die histologischen Veränderungen eines frühen LS unterscheiden sich jedoch stark von denen eines späten LS mit flach-atropher Epidermis, hochgradig sklerosierter Dermis mit starr-ektatischen Gefäßen. Die Epidermis eines frühen LS ist meist normal mit nur geringen Unregelmäßigkeiten der Reteleisten. Die Basalmembran ist normal oder nur fokal minimal verbreitert, die ödematöse Dermis zeigt nur vereinzelt ektatische Gefäße. Das oft sehr dichte lichenoide und intraepidermale Infiltrat erklärt die Spongiose und Vakuolisierung der basalen Keratinozyten. Extrem frühe Formen zeigen nur ein spärliches Lymphozyteninfiltrat und Hyper-/Parakeratose der infundibulären Ostien. Eine frühe lokale Therapie kann das Fortschreiten zum atrophen irreversiblen LS verhindern.

Abstract

Vulvar lichen sclerosus (LS) is a chronic progressive skin disease of unclear etiology. It is often overlooked in early stages, but progresses to destructive atrophy and is associated with an increased risk of vulvar squamous cell carcinoma. The classical symptoms are pruritus and pain, but they are often not distinctive, so that unclear vulvar problems often lead to a biopsy. The histological picture of early LS is quite different from that of late LS with an atrophic epidermis, markedly sclerotic dermis and stiff dilated vessels. The epidermis in early LS is usually normal with only minor irregularities in the rete pattern. The basement membrane is normal or focally widened, while the edematous dermis has only scattered ectatic vessels. The often dense lichenoid and intraepidermal infiltrate explains the spongiosis and vacuolization of the basal layer keratinocytes. Very early cases may only have a sparse lymphocytic infiltrate and hyper-/parakeratosis of the follicular ostia. Early topical therapy can dampen the progression to atrophic, irreversible LS.

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Danksagung

Die Autoren danken der Österreichischen Krebshilfe Steiermark für die Unterstützung dieser Publikation aus dem Projekt 04/2001.

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Regauer, S., Liegl, B., Reich, O. et al. Lichen sclerosus vulvae. Hautarzt 55, 158–164 (2004). https://doi.org/10.1007/s00105-003-0645-8

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