School of Dental Medicine, University of Zagreb, Croatian Dental Society - Croatian Medical Association
Abstract
Pemfigus vulgaris je rijetka, potencijalno smrtonosna kronična autoimuna vezikulobulozna bolest kože i sluznice. Zahvaća populaciju pedesetih i šezdesetih godina, a u edolescenciji je iznimno rijetka. Osamdeset do devedeset posto pacijenata oboljelih od pemfigus vulgarisa razvije oralne lezije, a kod šezdeset posto oralne su lezije prvi znak bolesti. Oralne lezije pojave se 4. mjeseca prije manifestacija na koži. Autori prikazuju slučaj pemfigus vulgarisa koji se inicijalno razvio u 18-godišnje djevojke i to samo na sluznici usta. Kliničkim i histopatološkim nalazima te izravnom i neizravnom imunofluorescencijom sluznice usta potvrđena je dijagnoza: pemfigus vulgaris, s pozitivnim antitijelima na dezmogline 1 i 3. Pacijentica je uspješno liječena sustavskim i topikalnim kortikosteroidima te mikonazolom. Tijekom 11 godina bolest je pod kontrolom s niskom dozom kortikosteroida i pacijentici se nikada nisu razvile promjene na koži. Prepoznati oralne lezije pemfigus vulgarisa od strane kliničara, postaviti ranu dijagnozu bolesti i odrediti adekvatnu terapiju važno je za smanjenje i eliminaciju intenziteta daljnje intraoralne i /ili ekstroralne zahvaćenosti bolešću.Pemphigus vulgaris is a rare, potentially lethal chronic autoimmune vesiculobullous disease of the skin and the mucous membrane. Most commonly it involves patients in their fifties and sixties and extremly rare in adolescence. Eighty to ninety percent of patient with pemphigus vulgaris develop oral lesions and 60% of cases oral lesions are the first sign. It is common for the oral lesions to be present up to 4 months before the skin lesions apper. The autors present a case of pemphigus vulgaris, which developed initially in an 18-year old girl and was confined to her mouth. Clinical and histopathological findings, also direct and indirect immunofluorescence studies of the oral mucosa confirmed the diagnosis of pemphigus vulgaris, with positive antibodies to desmoglein 3 and 1. The patient was successfully treated with systemic and topical corticosteroids in association with miconazole. The patient is treated with low-dose systemic corticosteroids, and during 11 years skin involvement has not been found. Recognition of oral lesions of adolescent pemphigus vulgaris by the clinician, its early diagnosis, and prompt therapy are important in order to eliminate or reduce the severity of further intraoral or extraoral involvement
