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Skleromyxödem

Scleromyxedema

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Zusammenfassung

Das Skleromyxödem ist eine sehr seltene Erkrankung, die gewöhnlich in Verbindung mit einer monoklonalen Gammopathie auftritt und systemische Symptome aufweisen kann. Die Pathogenese des Skleromyxödems ist unbekannt. Der klinische Verlauf ist chronisch und progressiv und kann zu schwerer Morbidität bzw. zum Tod führen. Der Hautbefund besteht aus multiplen wachsartigen Papeln und verhärteten Plaques. Eine fortschreitende Hautbeteiligung kann zu einer verminderten Beweglichkeit von Mund und Gelenken führen. Extrakutane Manifestationen können im Nerven-, muskuloskeletalen oder kardiovaskulären System, im Gastrointestinal- oder Atemtrakt oder an den Nieren auftreten. Eine für das Skleromyxödem zugelassene oder evidenzbasierte Therapie gibt es nicht. Als Therapie der ersten Wahl gelten hoch dosierte Immunglobuline, gefolgt von Lenalidomid (bzw. Thalidomid) und systemischen Glukokortikosteroiden, bei schweren Verläufen auch eine autologe hämatopoetische Stammzelltransplantation. In der Regel treten nach Absetzen einer erfolgreichen Therapie Rezidive auf, sodass eine engmaschige interdisziplinäre Anbindung ratsam ist.

Abstract

Scleromyxedema is a rare disorder that frequently affects multiple extracutaneous organ systems and is usually associated with monoclonal gammopathy. The pathogenesis of scleromyxedema is unknown. The clinical course is chronic and progressive and can lead to marked morbidity or death. The skin findings consist of multiple waxy papules and indurated plaques. Progressive skin involvement can lead to decreased mobility of the mouth and joints. Extracutaneous manifestations occur in the musculoskeletal or cardiovascular system, in the gastrointestinal or respiratory tract, or in the kidneys. There are no approved or evidence-based treatment options available for scleromyxedema. High-dose immunoglobulins are considered the treatment of choice, followed by lenalidomide (or thalidomide) and systemic glucocorticosteroids, or in severe cases even autologous hematopoetic stem cell transplantation. Long-term maintenance treatment is usually required and close clinical follow-up is necessary as recurrence of scleromyxedema is common after withdrawal of an effective therapy.

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Authors and Affiliations

  1. Abteilung für translationale Dermatoinfektiologie, Westfälische Wilhelms-Universität Münster, Von-Esmarch-Str. 58, 48149, Münster, Deutschland

    M. Neufeld & C. Sunderkötter

  2. Universitätsklinik und Poliklinik für Dermatologie und Venerologie, Universitätsklinikum Halle (Saale), Martin-Luther-Universität Halle-Wittenberg, Halle, Deutschland

    C. Sunderkötter & R. K. C. Moritz

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  1. M. Neufeld
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Correspondence to M. Neufeld.

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M. Neufeld, C. Sunderkötter und R.K.C. Moritz geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren. Alle Patienten, die über Bildmaterial oder anderweitige Angaben innerhalb des Manuskripts zu identifizieren sind, haben hierzu ihre schriftliche Einwilligung gegeben. Im Falle von nicht mündigen Patienten liegt die Einwilligung eines Erziehungsberechtigten oder des gesetzlich bestellten Betreuers vor.

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Neufeld, M., Sunderkötter, C. & Moritz, R.K.C. Skleromyxödem. Hautarzt 69, 916–921 (2018). https://doi.org/10.1007/s00105-018-4257-8

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