Morbus Castleman-assoziierter paraneoplastischer Pemphigus bei einer 16-jährigen Patientin

Der paraneoplastische Pemphigus ist eine seltene, lebensbedrohliche Autoimmunerkrankung, die klinisch durch meist ausgedehnte und therapierefraktäre Schleimhauterosionen und polymorphe Hautveränderungen charakterisiert ist. Wir berichten hier über eine 16-jährige Patientin mit isolierten oralen Erosionen, bei der zunächst ein Schleimhautpemphigoid vermutet wurde und es unter Therapie mit Prednisolon und Dapson zu einer deutlichen Befundbesserung kam. Allerdings entwickelte die Patientin einige Monate später eine massive respiratorische Insuffizienz infolge einer Bronchiolitis obliterans, sodass eine Lungentransplantation geplant wurde. Im Rahmen der vorbereitenden Diagnostik wurde ein unizentrischer, abdominell lokalisierter Morbus Castleman diagnostiziert, was schließlich zu der Diagnose eines paraneoplastischen Pemphigus mit Nachweis von Envoplakin-Autoantikörpern führte. Durch die Tumorresektion und anschließende Lungentransplantation konnte ein guter Allgemeinzustand wiederhergestellt werden bei anhaltender mukokutaner Remission.

• Publication year

  2024

• Venue

  Die Dermatologie

• Publication date

  2024-04-24

• Fields of study

  Not labeled

• Identifiers
  DOI 10.1007/s00105-024-05328-5PMID 38656540PMCID 11300659
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

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