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Lichtschutz bei Xeroderma pigmentosum

Light protection for xeroderma pigmentosum

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Zusammenfassung

Xeroderma pigmentosum ist eine seltene autosomal-rezessiv vererbte Genodermatose, die durch Mutationen in Genen, verantwortlich für die Reparatur UV-induzierter DNA-Schäden, verursacht wird. Die Erkrankung ist charakterisiert durch erhöhte UV-Lichtsensitivität und eine Poikilodermie, Augenbeteiligung und bei manchen Patienten durch ausgeprägte Sonnenbrände und neurologische Mitbeteiligung. Es besteht ein erhöhtes Risiko für die Entwicklung von Augen- und Hauttumoren an sonnenexponierten Arealen. Derzeit besteht keine kurative Therapie. Als protektive Maßnahme wird ein absoluter Lichtschutz vor allen UV-Strahlungen dringend empfohlen.

Abstract

Xeroderma pigmentosum is a rare autosomal recessive disorder which is caused by germinal mutations responsible for the repair of ultraviolet (UV) radiation-induced DNA lesions. It is characterized by hypersensitivity to UV radiation, poikiloderma, ocular surface disease, and in some patients pronounced sunburn and neurological disease. Patients have a very high risk of developing ocular and skin cancer on exposed body sites. No cure is available for these patients except complete protection from all types of UV radiation.

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Authors and Affiliations

  1. Universitätsklinik für Dermatologie, Franz-Josef-Strauß Allee 11, 93053, Regensburg, Deutschland

    M. Ettinger &  M. Berneburg

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  1. M. Ettinger
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  2. M. Berneburg
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Correspondence to M. Berneburg.

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Interessenkonflikt

M. Ettinger und M. Berneburg 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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Ettinger, M., Berneburg, M. Lichtschutz bei Xeroderma pigmentosum. Hautarzt 68, 359–363 (2017). https://doi.org/10.1007/s00105-017-3978-4

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