Pemphigus herpetiformis – prikaz bolesnika sa retkom formom pemfigusa i pregled literature

  • Jelena Stojković-Filipović Clinic of Dermatovenereology, Clinical Center of Serbia, Department of Dermatovenereology, Faculty of Medicine, University of Belgrade Belgrade, Serbia
  • Branislav Lekić Clinic of Dermatovenerology, Clinical Center of Serbia, Belgrade, Serbia
  • Danijela Milčić Clinic of Dermatovenerology, Clinical Center of Serbia, Belgrade, Serbia; Faculty of Medicine, University of Belgrade, Belgrade, Serbia
  • Mirjana V. Milinković Clinic of Dermatovenerology, Clinical Center of Serbia, Belgrade, Serbia; †Faculty of Medicine, University of Belgrade, Belgrade, Serbia
DOI: https://doi.org/10.2298/VSP150617044S
Ključne reči: pemphigus||, ||pemfigus, rare diseases||, ||retke bolesti, diagnosis||, ||dijagnoza, drug therapy||, ||lečenje lekovima, treatment outcome||, ||lečenje, ishod,

Sažetak


Uvod. Pemphigus herpetiformis predstavlja retku varijantu pemfigusa, sa karakterističnom kliničkom prezentacijom i patohistološkim nalazom koji se razlikuju od klasičnih formi pemfigusa, i imunološkim karakteristikama koje odgovaraju pemfigusu. Prikaz bolesnika. U radu je prikazana bolesnica stara 65 godina sa početnim pruritusom, a potom pojavom pruritičnih papula i plakova, sa mestimično anularno raspoređenim vezikulama na periferiji pojedinih lezija, na trupu i ekstremitetima. Na mukozama nije bilo patoloških promena. Patohistološkim pregledom utvrđena je spongioza i intraepidermalne vezikule u srednjim slojevima epiderma i supkornealno, uz ćelijski infiltrat sačinjen od neutrofila i eozinofila. Direktnom imunoflorescentnom mikroskopijom uočeni su intercelularni depoziti IgG autoantitela, izraženije u gornjim slojevima epiderma. Indirektnom imunoflorescentnom mikroskopijom u serumu bolesnika dokazana su autoantitela IgG klase. Bolesnica je inicijalno lečena opštom kortikosteroidnom terapijom i azatioprinom, ali je do kompletne kliničke remisije dovela terapija dapsonom. Zaključak. Od kada je ovaj entitet prvi put opisan pre 40 godina, u literaturi je prikazano oko 100 bolesnika.  Pemfigus herpetiformis je važno prepoznati s obzirom na drugačiju kliničku prezentaciju, tok bolesti i terapijski pristup u odnosu na konvencionalne forme pemfigusa.

 

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Objavljeno
2017/03/08
Broj časopisa
Vol. 73 Br. 10 (2016)
Rubrika
Prikaz bolesnika