Lezije oralnih i perioralnih mekih tkiva i oralne funkcije kod bolesnika sa distrofičnom buloznom epidermolizom
Sažetak
Uvod/Cilj. Distrofijska bulozna epidermoliza (DBE) karakteriše se izrazitim kožnim i sistemskim promenama, kao i mnogobrojnim oralnim manifestacijama. Cilj rada bio je da se ispita stanje oralnih i perioralnih mekih tkiva i funkcionalnost usne duplje bolesnika sa DBE praćenjem promena u ovim tkivima u periodu od godinu dana. Metode. Klinički su pregledana 24 bolesnika sa DBE (uzrasta od 1 meseca do 36 godina), prvi put (T0), posle 6 meseci (T6) i posle 12 meseci (T12). Praćeni su pojava i lokalizacija perioralnih i oralnih bula i ožiljaka, maksimalno otvaranje usta, prisutnost smanjene dubine vestibuluma, odsustvo kvržica (papila) jezika i nabora (rugae) nepca i ograničena pokretljivost jezika usled formiranja ožiljka. Vrednosti maksimalnog otvaranja usta pri prvom pregledu upoređene su sa vrednostima izmerenim u zdravoj kontrolnoj grupi istog uzrasta. Analizirani su i uzrast bolesnika i razlike između dominantnog i recesivnog oblika DBE. Rezultati. Prosečna vrednost maksimalnog otvaranja usta je bila značajno niža u bolesnika sa DBE u poređenju sa zdravim osobama. Bule i ožiljci na oralnoj i perioralnoj regiji, mikrostomija i smanjen vestibulum bili su veoma često prisutni, bez statistički značajne razlike između T0, T6 i T12 pregleda. Učestalost ograničene pokretljivosti jezika i odsustvo papila jezika i nabora nepca su se u periodu od godinu dana statisitički značajno povećavali. Bolesnici sa mikrostomijom, smanjenim vestibulumom i ograničenom pokretljivošću jezika zbog formiranja ožiljka bili su značajno stariji u odnosu na bolesnike bez tih promena. Papile jezika i nabori nepca su bili značajno češće odsutni kod recesivnog u odnosu na dominantni oblik DBE. Zaključak. DBE dovodi do značajnih promena u oralnom i perioralnom mekom tkivu i poremećaja oralnih funkcija.
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