Zusammenfassung
Bei dem bisher gesunden Jungen kam es im Rahmen eines benignen Infekts zu rasch fortschreitenden Hautnekrosen bei gutem Allgemeinzustand. Prädisponierend hierfür waren prothrombotische Risikofaktoren (Faktor-V-Leiden-Mutation G1691A, erhöhtes Lipoprotein a), deren Vorliegen bei diesem Patienten bislang unbekannt gewesen war. Auslösend für die Erkrankung war ein zusätzlicher, postinfektiös erworbener Mangel an Protein S und C. Postinfektiöse Vaskulitiden werden am häufigsten nach Infektionen durch Varizellen oder Streptokokken gesehen. Im vorliegenden Fall war die Vorgeschichte bis auf eine vor kurzem abgelaufene Influenzainfektion unauffällig.
Abstract
This case report describes a previously healthy boy who developed rapidly progressing necrotic skin lesions after a mild upper airway infection. Some prothrombotic risk factors [factor V Leiden mutation G1691A, elevated lipoprotein(a)], which had not been known to the patient and his family before, were detected. In addition, a postinfectiously acquired protein C and S deficiency triggered the purpura fulminans. Postinfectious vasculitis is most commonly seen in children after varicella or streptococcal infections. Apart from antibodies showing a recent influenza infection, no other evidence of an infection could be found.
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Creutzfeldt, R., Jansson, A. & Kurnik, K. Rasch fortschreitende Hautnekrosen. Monatsschr Kinderheilkd 158, 115–117 (2010). https://doi.org/10.1007/s00112-008-1845-1
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DOI: https://doi.org/10.1007/s00112-008-1845-1