Subcorneal pustular dermatosis: clinical characteristics and long-term follow-up of seventeen patients

Tatjana Radević; Željko P. Mijušković; Lidija  Kandolf

doi:10.2298/VSP230830064R

Tatjana Radević Military Medical Academy, Department of Dermatology and Venereology, Belgrade, Serbia; University of Defence, Faculty of Medicine of the Military Medical Academy, Belgrade, Serbia
Željko P. Mijušković Military Medical Academy, Department of Dermatology and Venereology, Belgrade, Serbia; University of Defence, Faculty of Medicine of the Military Medical Academy, Belgrade, Serbia
Lidija Kandolf Military Medical Academy, Department of Dermatology and Venereology, Belgrade, Serbia; University of Defence, Faculty of Medicine of the Military Medical Academy, Belgrade, Serbia

DOI: https://doi.org/10.2298/VSP230830064R

Ključne reči: biopsija;, dapson;, dijagnoza;, imunohistohemija;, koža, vezikulobulozne bolesti;, lečenje, ishod

Sažetak

Uvod/Cilj. Subkornealna pustulozna dermatoza (SPD) je retko, relapsirajuće vezikulopustulozno oboljenje, koje predominantno zahvata fleksorne površine. S obzirom da su u dostupnoj literaturi podaci o toku i trajanju SPD veoma oskudni, cilj rada bio je da se analiziraju kliničke karakteristike i rezultati dugotrajnog praćenja bolesnika sa SPD. Metode. Pretraženi su medicinski kartoni iz bolničke baze podataka bolesnika sa SPD hospitalizovanih u periodu od 1985. do 2014. godine. Fokus je bio na kliničkim karakteristikama, pridruženim bolestima, toku bolesti i ishodima. Rezultati. Analizirano je 17 bolesnika sa kliničkim karakteristikama SPD: devet žena i osam muškaraca čija prosečna starost u trenutku manifestacije bolesti je bila 45 godina (raspon 18–90 godina). Podaci o praćenju bolesti bili su dostupni za 12 bolesnika. Srednje vreme praćenja iznosilo je 9,5 godina (1–28 godina). U cilju uspostavljanja histološke dijagnoze bile su potrebne ponovljene biopsije (u proseku 1,7 biopsija po bolesniku). Kod jednog bolesnika je direktnom imunofluorescencijom dijagnostikovan IgA pemfigus. Kod većine bolesnika kožna erupcija bila je rasprostranjena, dok su kod manjeg broja bolesnika promene bile prisutne samo na fleksornim površinama. Disproteinemija bila je prisutna kod tri bolesnika. Bolest je bila samoograničavajuća kod tri bolesnika; pet bolesnika je imalo blage relapse 1–3 puta godišnje, bez potrebe za lečenjem; četiri bolesnika imala su kontinuirane relapse, koji su zahtevali lečenje. Većina bolesnika sa SPD je pozitivno reagovala na dapson. Zaključak. Bolest SPD je retka, a obično se javlja u petoj deceniji života. Mada je kod nekih bolesnika samoograničavajuća, kontinuirana terapija je potrebna približno jednoj trećini bolesnika zbog čestih relapsa.

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Subkornealna pustulozna dermatoza: kliničke karakteristike i dugotrajno praćenje sedamnaest bolesnika

Sažetak

Reference