Pemfigoid pęcherzowy to przewlekła choroba skórna o podłożu autoimmunologicznym. Zapadają na nią głównie osoby po 65. roku życia. Jej charakterystycznym objawem są napięte pęcherze surowicze i krwiste, którym towarzyszy uporczywy świąd.
Pemfigoid pęcherzowy uznawany jest za najczęstsze podnaskórkowe schorzenie pęcherzowe o podłożu autoimmunologicznym. Najwięcej przypadków pemfigoidu wykrywa się u osób po 80. roku życia, u których występują choroby współistniejące, takie jak nadciśnienie, schorzenia układu sercowo-naczyniowego czy cukrzyca. Z tego powodu metody leczenia pemfigoidu mogą być uzależnione od przyjmowanych już przez pacjenta leków. Pełne wyleczenie może trwać około roku.
Przyczyny pemfigoidu pęcherzowego
Zmiany pęcherzowe powstają wskutek dysfunkcji układu odpornościowego. Obecne w surowicy chorego autoprzeciwciała działają przeciw antygenom błony podstawnej łączącej naskórek ze skórą właściwą. W wyniku połączenia się przeciwciał i antygenów błony podstawnej dochodzi do oddzielenia się naskórka od skóry właściwej, co powoduje wystąpienie pęcherzy.
Pemfigoid pęcherzowy może rozwinąć się na skutek przyjmowania niektórych leków, takich jak diazepam, diuretyki, furosemid, penicylina czy inhibitory ACE. Objawy mogą zostać wywołane przez stosowanie preparatów miejscowych w terapii świerzbu czy łuszczycy, m.in. esteru benzylowego kwasu benzoesowego czy kwasu salicylowego. W przypadku gdy pemfigoid pęcherzowy został wywołany przez leki, zaleca się ich odstawienie. Wykwity powinny wówczas ustąpić samoistnie.
W niektórych przypadkach wystąpienie pemfigoidu jest powiązane z chorobą nowotworową, zwykle rakiem trzustki, płuc, układu pokarmowego lub moczowego. Pemfigoid może wystąpić także pod wpływem promieniowania UV.
Zmiany skórne charakterystyczne dla pemfigoidu pęcherzowego
Pierwszymi zmianami skórnymi w przebiegu pemfigoidu są rumienie obrzękowe i pokrzywkowate. Wywołują nasilony świąd i pieczenie. Następnie pojawiają się napięte pęcherze krwiste lub surowicze. Mogą być umiejscowione na skórze niezmienionej lub objętej rumieniem. Zwykle zlokalizowane są na tułowiu, w zgięciach kończyn i na błonie śluzowej jamy ustnej. Po zmianach mogą pozostać przebarwienia pozapalne. U 20 proc. chorych na błonach śluzowych występują nadżerki, czyli płytkie owrzodzenia. Zmianom skórnym często towarzyszą objawy ogólne, takie jak wysoka gorączka, pogorszenie samopoczucia, utrata apetytu i zmęczenie.
Leczenie zmian skórnych
Terapia pemfigoidu wymaga wdrożenia działań wspólnie uzgodnionych przez dermatologa i lekarza internistę z uwzględnieniem chorób współistniejących pacjenta. Leczenie obejmuje miejscową terapię zmian skórnych, zmniejszenie uczucia świądu i zapobieganie nawrotom.
Pęcherze przekłuwa się w sterylnych warunkach. Podczas otwierania pęcherzy nie należy usuwać ich pokrywy, ponieważ stanowi naturalną warstwę ochronną. Po nakłuciu zmian pęcherzowych przemywa się je środkami antyseptycznymi. Następnie stosuje się miejscowo leki glikokortykosteroidowe. Wysokość dawki ustalana jest podczas wizyt kontrolnych w zależności od nasilenia zmian skórnych. Jeżeli zmiany nie są rozległe, lekarz może zalecić przyjmowanie antybiotyków, m.in. tetracyklinę lub erytromycynę. Jeśli objawy są nasilone, wdraża się leki immunosupresyjne.
Po ustąpieniu zmian skórnych zaleca się dalsze monitorowanie stanu pacjenta. Chory powinien kontynuować wizyty kontrolne w poradni dermatologicznej, ponieważ pemfigoid ma tendencję do wielokrotnych nawrotów. Najwcześniejsze symptomy nawrotu to świąd, wystąpienie zmian rumieniowych i wydrapań.
Źródła: „Pemfigoid – diagnostyka i leczenie. Konsensus Polskiego Towarzystwa Dermatologicznego”, w: „Przegląd Dermatologiczny”, 2016, 103, 19–34
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Przeczytaj także: Pęcherzyca – przewlekła choroba skóry. Jak ją leczyć?
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