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Scleroedema adultorum

Klinik, Diagnostik, Differenzialdiagnose, therapeutisches Vorgehen

Scleredema adultorum

Clinical presentation, diagnostic workup, differential diagnosis, treatment option

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Zusammenfassung

Das Scleroedema adultorum (SA) ist eine seltene skleromuzinöse Erkrankung nicht geklärter Ätiologie, die charakteristischerweise zu brettharten Indurationen im Bereich des Nackens und oberen Rumpfes führt. Drei Typen des SA werden entsprechend ihrer Assoziation zu vorbestehenden bzw. zugrunde liegenden Erkrankungen unterschieden: SA bei Infektionen (v. a. Streptokokkeninfektionen der oberen Atemwege), SA bei monoklonaler Gammopathie und SA bei Diabetes mellitus. Die klinische Ausprägung, Schwere der Erkrankung und Verlauf variieren je nach Subtyp stark. Es existieren bisher keine evidenzbasierten Therapiestandards für das SA. Während spontane Remissionen häufig bei Infekt-assoziierten Formen des SA auftreten, kommt es beim SA im Rahmen von Diabetes mellitus oder monoklonaler Gammopathie in der Regel zu einem chronisch progredienten Verlauf. Zu den derzeitig empfohlenen Erstlinientherapien zählen Phototherapie und Methotrexat, eine klinische Besserung zeigt sich oftmals aber erst nach mehreren Monaten, und Therapieversagen ist häufig. Ergänzend sollte bei allen Formen des SA auch eine Physiotherapie zur Verbesserung der Beweglichkeit erfolgen.

Abstract

Scleredema adultorum (SA) is a rare scleromucinous disease of unknown etiology that characteristically leads to wodden induration located on the neck and upper trunk. Three types of SA can be distinguished according to the association with pre-existing or underlying disease: SA in association with infections (mostly streptococcal infections of the upper respiratory tract), SA in association with monoclonal gammopathy, and SA in association with diabetes. The clinical findings, extent of disease, and course substantially differ depending on the subtype of SA. Spontaneous regression often occurs in infection-associated SA, whereas patients with diabetes or monoclonal gammopathy usually show a chronic progressive course of disease. Phototherapy and methotrexate are the current recommended first-line treatments for SA, clinical improvement often takes several months, and treatment failure is frequent. Physiotherapy should be offered in all types of SA in order to improve motility.

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  1. Klinik für Dermatologie, Venerologie und Allergologie, HELIOS St. Elisabeth Klinik Oberhausen, Universität Witten/Herdecke, Josefstr. 3, 46045, Oberhausen, Deutschland

    A. Kreuter

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Dieser Beitrag beinhaltet keine vom Autor 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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Kreuter, A. Scleroedema adultorum. Hautarzt 69, 908–915 (2018). https://doi.org/10.1007/s00105-018-4259-6

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