Zusammenfassung
Die lokalisierte (oder zirkumskripte) Sklerodermie (LS) oder Morphea ist eine sklerosierende Bindegewebserkrankung der Haut, die auch darunterliegende Gewebe wie Subkutis, Muskulatur und Skelett betreffen kann. Viele Patienten weisen extrakutane Symptome und antinukleäre Antikörper auf. Ein Übergang in eine systemische Sklerose wird jedoch nicht beobachtet. Die LS beginnt oft im Kindesalter und zeigt ein breites klinisches Spektrum. Der lineäre Subtyp ist dabei die häufigste Form und zeichnet sich durch Progredienz und ein erhebliches Komplikationsrisiko aus. Die Erkrankung kann über Jahre fortschreiten und insbesondere während des Körperwachstums zu schweren funktionellen und ästhetischen Einbußen führen. Die Ursache der LS ist nicht geklärt. Ein genetischer Hintergrund wird vermutet, und Triggerfaktoren wie Traumen, Impfungen und Infektionen werden diskutiert, die sekundäre Immunphänomene auslösen. Die LS bleibt oft lange unerkannt, was zu einer Therapieverzögerung führt. Für die progrediente (meist lineäre) LS ist heute die Kombination von systemischen Kortikosteroiden und Methotrexat die Therapie der Wahl, während für oberflächliche lokalisierte Formen bei Jugendlichen die Lichttherapie (UVA-1 oder „UVB-narrow band“) geeignet ist.
Abstract
Localized scleroderma or morphea is a sclerosing connective tissue disease of the skin, which may affect underlying tissues such as subcutis, muscle and bone. Many patients show extracutaneous symptoms and antinuclear antibodies, however, secondary transformation into systemic sclerosis does not occur. Localized scleroderma usually begins in childhood with a wide variation in its clinical spectrum. The linear variant is the most common subtype in children, associated with a progressive course and increased risk of complications. The disease may progress over years and result in severe functional and cosmetic disability. The etiology of localized scleroderma remains unknown. A genetic background is suspected, while triggers such as trauma, vaccinations and infections may lead to secondary immunologic phenomena. Localized scleroderma often remains unrecognized for a long time, resulting in substantial delay in treatment. The combination of systemic corticosteroids and methotrexate has been established as first-line therapy for progressive (usually linear) disease, whereas phototherapy (UVA-1 or UVB-narrow band) is suitable for adolescents with superficial circumscribed subtypes.
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Weibel, L. Lokalisierte Sklerodermie (Morphea) im Kindesalter. Hautarzt 63, 89–96 (2012). https://doi.org/10.1007/s00105-011-2199-5
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DOI: https://doi.org/10.1007/s00105-011-2199-5
Schlüsselwörter
- Morphea
- Zirkumskripte/lokalisierte Sklerodermie
- Sklerodermie en coup de sabre
- Parry-Romberg-Syndrom
- Methotrexat