Pemphigus foliaceus

Zuletzt aktualisiert: 2022-11-16

Autor(en): Anzengruber F., Navarini A.

ICD11: EB40.1

Anti-DSG1 Autoimmunkrankheit

Am häufigsten im mittleren Alter, es wurde aber auch von 18 Monaten bis 70 Jahren Lebensalter beschrieben. 

  • Klassische Variante
  • Pemphigus erythematosus Senear Usher

Autoimmunreaktion mit Antikörpern gegen DSG1

Häufig nicht als Bullae perzeptible, erythematöse, teils erosive, fein schuppende, auf die seborrhoischen Zonen fokussierte Areale. Kann ähnlich aussehen wie ein Ekzem. Kann jucken und brennen. Typisch ist der Beginn nach emotionalem Stress (33% der Fälle), ansonsten Risikofaktoren Sonnenlicht, Hitze. 

 

Biopsie mit DIF

Anti-DSG1,3

Diagnostik für Myasthenia gravis und Thymom

Seborrhoische Zonen, kann aber den ganzen Körper betreffen. Geht nicht auf die Schleimhäute.

Brennende und juckende Blasen entwickelt, mechanisch? Auslöser Stress? 

Muskelschwäche?

Superfizielle, intraepidermale Bullae. In den Hohlräumen ist nichts, oder Fibrin, oder auch Neutrophile. Dyskeratotische Zellen im Str. granulosum sind hinweisend auf Pemphigus foliaceus. Früh kann auch eine eosinophile Spongiose auftreten, dann Achtung Differentialdiagnose bullöses Pemphigoid, Pemphigus vulgaris, Dermatitis herpetiformis. 

DIF in fast 100% positiv, interzelluläre Immunglobuline und Komplement in der Epidermis. 

Impetiginisierung.

Keine möglich.

Relativ gut, kann punktuelle Rezidive geben.

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