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Aktuelle Dermatologie 2014; 40(05): 171-176
DOI: 10.1055/s-0034-1365520
Eine Klinik im Blickpunkt
© Georg Thieme Verlag KG Stuttgart · New York

Scleroedema adultorum – Eine Übersicht

Scleroderma adultorum – An Overview
J. Kirschke
Klinik für Dermatologie, Venerologie und Allergologie, HELIOS St. Elisabeth Klinik, Oberhausen
,
A. Kreuter
Klinik für Dermatologie, Venerologie und Allergologie, HELIOS St. Elisabeth Klinik, Oberhausen
› Author Affiliations
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Publication History

Publication Date:
10 April 2014 (online)

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Zusammenfassung

Das Scleroedema adultorum ist eine sklerosierende Hauterkrankung, die meistens in Assoziation zu Diabetes mellitus, Infektionen (vor allem Streptokokken-Infektionen der oberen Atemwege) oder monoklonaler Gammopathie auftritt. Klinisch zeigen sich symmetrische, brettharte Hautindurationen, die initial typischerweise im Bereich des oberen Rumpfes und Nackens sowie bei postinfektiösen Formen im Gesichts- und Halsbereich auftreten. Der klinische Verlauf variiert je nach Subtyp stark. Spontane Remissionen treten häufig bei Streptokokken-assoziierten Formen auf, während die Erkrankung bei Patienten mit Diabetes mellitus oder monoklonaler Gammopathie häufig einen chronisch progressiven Verlauf nimmt. Die Diagnose wird durch Anamnese, körperliche Untersuchung und Hautbiopsie gestellt. Serologische Untersuchungen sind sinnvoll, um assoziierte Erkrankungen nachzuweisen. Es existieren bisher keine evidenzbasierten Therapiestandards. Eine physikalische Therapie sollte allen Patienten zur Verbesserung der Beweglichkeit angeboten werden. Derzeitig ist die Fototherapie, insbesondere die UVA1-Fototherapie, in vielen Zentren die Therapie der ersten Wahl. Alternativ kann auch die PUVA-Therapie oder Schmalband-UVB mit 311 nm als Behandlungsoption in Erwägung gezogen werden. Auch der Einsatz von Immunsuppressiva oder Strahlentherapie mit schnellen Elektronen wurde bei Scleroedema adultorum beschrieben, sollte jedoch nur bei Patienten mit schwerem Verlauf und Kontraindikationen gegen eine Fototherapie in Erwägung gezogen werden.

Abstract

Scleroedema adultorum is a sclerotic skin disease that predominantly occurs in association with diabetes, infections (mostly streptococcal-infections of the upper respiratory tract) or monoclonal gammopathy. The disease is clinically characterized by a symmetric, wooden induration of the skin that typically affects the upper trunk and neck or the facial and throat area in case of infection-associated types. The clinical course substantially differs depending on the subtype of disease. Spontaneous regression often occurs in streptococcal-associated types of disease, whereas scleroedema in patients with diabetes or monoclonal gammopathy usually show a chronic progressive course of disease. The diagnosis of scleroedema is made by anamnesis, skin examination, and biopsy. Serological examinations are useful to screen for associated diseases. To date, no evidence-based treatment recommendations exist. Physiotherapy should be offered to all patients in order to improve motility. Phototherapy, especially UVA1-phototherapy, is the first choice of treatment in many centers. Alternatively, PUVA-therapy or narrow-band-UVB with 311 nm might be considered. Immunsuppressive drugs or radiotherapy have been reported for scleroedema as well, but might be only considered for patients with a severe course of disease or contraindications against phototherapy.

 

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