Sklerodermie – „die harte Haut“

Zusammenfassung

Unter dem Begriff Sklerodermie ist eine heterogene Gruppe von Erkrankungen zusammengefasst, die eine kutane Sklerose gemeinsam haben. Die einzelnen Erkrankungen variieren in der Beteiligung betroffener Strukturen wie Haut, Fettgewebe, Muskulatur, Gelenkstrukturen, Knochen, innerer Organe und der damit einhergehenden Krankheitsausprägung. Es lassen sich grundsätzlich zwei verschiedene Subtypen unterscheiden: die lokalisierte Sklerodermie (LoS) und die systemische Sklerose (SSc), die sich jeweils in weitere Subgruppen unterteilen lassen. Es handelt sich um zwei verschiedene Erkrankungen, die sich in der Ausprägung der Hautsklerosierung, der Beteiligung innerer Organe, im Antikörper-Profil, im Krankheitsmanagement sowie der Prognose unterscheiden. Darüber hinaus gibt es Krankheitsbilder, die eine Sklerodermie imitieren und daher als sklerodermie-artige Erkrankungen oder auch als Pseudosklerodermien bezeichnet werden. Um die richtige Diagnose stellen zu können, ist ein standardisiertes diagnostisches Vorgehen bedeutsam. Die frühe Diagnosestellung ist wichtig, um frühzeitige eine Therapie einleiten zu können und so funktionelle kutane und extrakutane Einschränkungen und auch kosmetische Schäden gering halten zu können.

Abstract

The term scleroderma covers a heterogeneous group of diseases sharing the characteristic feature of cutaneous sclerosis. The individual diseases vary in the involvement of affected structures such as skin, adipose tissue, muscles, joint structures, bones, internal organs and the associated disease expression. Basically, two different subtypes can be distinguished: localised scleroderma (LoS) and systemic sclerosis (SSc), each of which are subdivided into further subgroups. These are two distinct diseases that differ in the extent of skin sclerosis, involvement of internal organs, antibody profile, disease management and prognosis. In addition, there are clinical pictures that mimic scleroderma and are therefore referred to as scleroderma-like diseases or pseudoscleroderma. In order to make the correct diagnosis, a standardised diagnostic procedure is significant. Early diagnosis is important in order to initiate early treatment and thus minimise functional cutaneous and extracutaneous impairment as well as cosmetic damage.

Schlüsselwörter

Sklerodermie - lokalisierte Sklerodermie - zirkumskripte Sklerodermie - Morphea - systemische Sklerose

Keywords

scleroderma - systemic sclerosis - circumscribed scleroderma - morphea - localised scleroderma

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