Pemfigus i pemfigoid s osvrtom na oralnu sluznicu

master's thesis

• Title (english): Pemphigus and pemphigoid with reference to the oral mucosa
• Author: Simone Gilli
• Mentor: Božana Lončar Brzak (mentor)
• Committee member: Božana Lončar Brzak (predsjednik povjerenstva)
• Committee member: Ana Andabak Rogulj (član povjerenstva)
• Committee member: Ivana Škrinjar (član povjerenstva)
• Granter: University of Zagreb; School of Dental Medicine; (Department of Oral Medicine); Zagreb
• Defense date and country: 2022-09-15, Croatia
• Scientific / art field, discipline and subdiscipline: BIOMEDICINE AND HEALTHCARE; Dental Medicine; Oral Medicine

Abstract

Pemfigus i pemfigoid su potencijalno smrtonosne autoimune bulozne bolesti koje mogu zahvatiti sluznice i kožu. Dijagnoza se postavlja na temelju laboratorijskih i histoloških pretraga, kliničkog pregleda te anamneze. Patohistološki je pemfigus obilježen akantolizom tj. pucanjem međustaničnih veza (dezmosoma) koje se nalaze u trnastom sloju epitela zbog stvaranja antitijela usmjerenih prema tim spojevima. Posljedica toga je nastanak intraepitelnog mjehura. Kod pemfigoida su protutijela usmjerena protiv hemidezmosoma, multiproteinskih kompleksa koji se nalaze duž bazalne membrane i spajaju epitel s vezivom, te je prisutan nalaz subepitelnog mjehura. Nakon pucanja mjehura nastaju erozije, lezije koje se mogu inficirati ukoliko nije ordinirana lokalna i/ili sustavna terapija koja smanjuje upalu i imunološku reakciju, ublažava simptome i potiče epitelizaciju sluznice. Zlatni standard za detekciju bolesti je direktna imunofluorescencija koja se provodi uzimanjem uzorka iz nepromijenjenog perilezijskog tkiva. Kako bi se potvrdila dijagnoza ovih autoimunih bolesti tkivo ne smije biti razoreno te mora sadržavati u sebi depozite imunoglobulina G i/ili C3 komponentu komplementa na odgovarajućoj lokalizaciji, ovisno o tome koji je ciljni antigen. Uz
patohistološku analizu i direktnu imunofluorescenciju tkiva moguće je koristiti indirektnu imunofluorescenciju za praćenje tijeka bolesti te ELISA-u (engl. enzyme-linked immunosorbent assay), visoko osjetljivi i specifični imunoenzimski test. Pravodobna
dijagnostika te odgovarajuća terapija uvelike utječe na prognozu i krajnji ishod bolesti. Primarno liječenje pemfigusa su sistemski kortikosteroidi u kombinaciji s adjuvantnim imunosupresivima ili rituksimab (anti-CD20 monoklonalno antitijelo) dok se u teškim
slučajevima mogu primijeniti i intravenski imunoglobulini i plazmafereza. Liječenje pemfigoida obično uključuje topikalnu primjenu sredstva za ublažavanje bolova s protuupalnim djelovanjem, a za tu svrhu odlični su lokalni kortikosteroidi. Mogu se propisati i pomoćni imunosupresivni lijekovi. Novije metode liječenja i jasnije razumijevanje patogeneze bolesti poboljšali su prognozu pemfigusa/pemfigoida te smanjili mortalitet.

Abstract (english)

Pemphigus and pemphigoid are potentially fatal autoimmune diseases with blisters that affect mucocutaneous surfaces and skin. The diagnosis is made on the basis of laboratory and histopathological tests, clinical examination and medical history. Histopathologically, pemphigus is characterized by acantholysis i.e. the breaking of intercellular connections (desmosomes) located in the spinous layer of the epithelium due to the formation of antibodies directed towards these connections. The consequence of these events is the formation of an intraepithelial blister. In pemphigoid the antibodies are directed against hemidesmosomes, multiprotein complexes that are located along the basement membrane and connect the epithelium with the connective tissue. There is a finding of a subepithelial blister. After the bursting of the blisters, erosions occur, lesions that can become infected if supportive antiseptic therapy with agents that stimulate epithelialization of the mucous membrane has not been conducted. The gold standard for the detection of the disease is direct immunofluorescence,
which is performed by taking a sample from the unchanged perilesional tissue. In order to confirm the diagnosis of these autoimmune diseases, the tissue must not be necrotic and must contain deposits of immunoglobulin G and/or the C3 component of complement in the appropriate localization, depending on which antigen is affected. In addition to
pathohistological analysis and direct immunofluorescence of the tissue, it is possible to use indirect immunofluorescence to monitor the course of the disease and ELISA, enzyme-linked immunosorbent assay, a highly sensitive and specific immunoenzymatic test. Timely diagnosis and appropriate therapy greatly influence the prognosis and the final outcome of the disease. The primary treatment of pemphigus are systemic corticosteroids in combination with adjuvant immunosuppressants or rituximab (anti-CD20 monoclonal antibody), while in severe cases intravenous immunoglobulins and plasmapheresis can also be used. Treatment of pemphigoid usually involves the topical application of a pain reliever with an anti-inflammatory effect, and topical corticosteroids are excellent for this purpose. Auxiliary immunosuppressive drugs may also be prescribed. Newer treatment methods and a clearer understanding of the pathogenesis of the disease have improved the prognosis of pemphigus/pemphigoid and reduced mortality.

• Keywords: oralne bolesti; pemfigus; pemfigoid; kortikosteroid
• Keywords (english): Mouth Diseases; Pemphigous; Bullous Pemphigoid; Corticosteroid
• Language: croatian
• URN:NBN: urn:nbn:hr:127:415431
• Study programme: Title: dental medicine; Study programme type: university; Study level: integrated undergraduate and graduate; Academic / professional title: doktor/doktorica dentalne medicine (doktor/doktorica dentalne medicine)
• Type of resource: Text
• File origin: Born digital
• Access conditions: Open access
• Created on: 2022-10-25 08:53:27