Lichen sclerosus im Kindes- und Jugendalter

ZUSAMMENFASSUNG

Lichen sclerosus ist eine chronisch-entzündliche Erkrankung der Haut und Schleimhäute, die insbesondere im Anogenitalbereich, aber auch anderen Körperregionen auftreten kann. Die Ätiopathogenese ist bis heute nur unzureichend bekannt und vermutlich multifaktoriell. Im Vordergrund stehen immunologische und genetische Faktoren. Die ersten Symptome sind meistens unspezifisch: Juckreiz, Brennen, Schmerzen, Missempfindungen und Entzündung. Es kommt typischerweise im Verlauf zu Hautveränderungen der Anogenitalregion, die neben weißlicher Verfärbung und abnehmender Elastizität zu Narbenbildungen und einer veränderten Anatomie der Vulva führen kann. Langjähriger, unbehandelter vulvärer Lichen sclerosus ist außerdem ein Risikofaktor für die spätere Entstehung eines Vulvakarzinoms. Häufig kommt es aufgrund der unspezifischen Symptome im frühen Krankheitsstadium zu einer prolongierten Diagnosestellung. Die frühzeitige Einleitung einer spezifischen Therapie ist von wesentlicher Bedeutung, um zum einen die teils quälenden Symptome zu lindern und zum anderen den Langzeitfolgen entgegenzuwirken. Hier steht an erster Stelle die Therapie mit ultrapotenten Glukokortikoiden.

ABSTRACT

Lichen sclerosus is a chronic mucocutaneous inflammatory disease, which is predominantly located at the anogenital region, but can also appear at other body regions. The etiopathogenesis is still insufficiently known and probable multifactoriell. The first symptoms are mostly not specific: itching, burning sensation, pain, discomfort and inflammation. In the further course, skin alteration of the perianal region occurs. Beside demarcated white skin lesions and loss of elasticity, scarring and a distorted vulvar architecture can arise. Longterm, untreated vulvar lichen sclerosus is a risk factor for vulvar cancer. Because of the unspecific symptoms in the early stage of the disease, the diagnosis is often prolonged. The early beginning of a specific therapy is very important: to alleviate the excruciating symptoms on the on hand and to prevent complications on the other hand. The golden standard is the therapy with ultrapotent topical corticosteroids.

Schlüsselwörter

Lichen sclerosus - Kinder - Jugendliche

Key words

Lichen sclerosus - children - adolescent

Publication History

Article published online:
01 December 2023

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