Nekrotyczny rumień wędrujący objawia się rozległymi wykwitami i pęcherzami, na których tworzą się strupy. W obrębie zmian skórnych może dojść do nadkażeń bakteryjnych i grzybiczych. Schorzenie to w większości przypadków występuje w przebiegu glukagonomy.
Nekrotyczny rumień wędrujący niekiedy jest mylnie diagnozowany jako pęcherzyca liściasta lub toksyczna nekroliza naskórka. Tymczasem właściwe rozpoznanie tej dermatozy niejednokrotnie pozwala wykryć rozwój nowotworu neuroendokrynnego. Tym samym umożliwia to podjęcie leczenia onkologicznego jeszcze przed wystąpieniem przerzutów. Jak rozpoznać zmiany skórne w przebiegu nekrotycznego rumienia wędrującego?
Objawy nekrotycznego rumienia wędrującego
Na skórze chorego pojawiają się rozległe zmiany rumieniowo-wysiękowe. Wykwity występują głównie na częściach ciała szczególnie narażonych na ucisk lub tarcie. Są to m.in. okolice wyprzeniowe, takie jak pachwiny i doły pachowe. Wykwity mogą objąć także doły podkolanowe, okolice krocza, pośladki, podbrzusze, podudzia oraz twarz.
Zmiany postępują i szerzą się obwodowo. Wykwity mają tendencję do zlewania się i zajmowania większych obszarów. Ich rozprzestrzenianiu się towarzyszy nasilone złuszczanie naskórka. Na początku zmiany zazwyczaj mają postać silnie rumieniowatych, odgraniczonych blaszek lub plam o nieregularnych krawędziach. Następnie na ich powierzchni tworzą się pęcherze, które pokrywają się strupami. Po wygojeniu na skórze pozostają brązowawe przebarwienia. Zmianom mogą towarzyszyć nasilony świąd oraz ból.
U około 30 proc. pacjentów wykwitom skórnym towarzyszą zmiany skórno-śluzówkowe, takie jak kątowe zapalenie warg, żywoczerwone zabarwienie języka oraz zapalenie jamy ustnej. Rzadziej występują zaburzenia w obrębie paznokci, np. zanokcica i rozszczepienie paznokcia. W niektórych przypadkach rozwija się także łysienie rozlane.
Powikłania nekrotycznego rumienia wędrującego
Rozdrapywanie swędzących zmian może doprowadzić do powstania nadżerek. Podrażnione wykwity często ulegają zakażeniom. W większości przypadków dochodzi do infekcji spowodowanych przez bakterie gronkowca złocistego (Staphylococcus aureus) oraz drożdżaka bielnika białego (Candida albicans).
Nekrotyczny rumień wędrujący objawem innych chorób
W około 80 proc. przypadków nekrotyczny rumień wędrujący występuje u pacjentów chorujących na glukagonomę. Jest to rzadki neuroendokrynny nowotwór pochodzący z komórek alfa wysp trzustkowych. W przebiegu glukagonomy dochodzi do nadmiernego wydzielania glukagonu. U chorych oprócz nekrotycznego rumienia wędrującego pojawiają się m.in. cukrzyca o lekkim przebiegu, niedokrwistość, zakrzepica żylna i zatory płucne.
Nekrotyczny rumień wędrujący może również towarzyszyć marskości wątroby lub zapaleniu trzustki.
Leczenie nekrotycznego rumienia wędrującego
Jeżeli nekrotyczny rumień wędrujący towarzyszy glukagonomie, przeprowadza się chirurgiczne usunięcie guza pierwotnego nowotworu trzustki. Po zabiegu w większości przypadków dermatoza ustępuje w ciągu tygodnia. Zmiany mają jednak tendencję do nawracania. Nekrotyczny rumień wędrujący zazwyczaj jest objawem zaawansowanego stadium choroby, dlatego u pacjentów może dojść do przerzutów. Wówczas zmiany skórne nawracają. Z tego powodu zaleca się badania kontrolne obejmujące m.in. monitorowanie poziomu stężenia glukagenu.
W przypadku pacjentów, którzy nie kwalifikują się do operacji, wdraża się leczenie zachowawcze. W terapii stosuje się leki z somatostatyną, która obniża poziom glukagonu i insuliny. W większości przypadków powoduje to znaczne złagodzenie objawów skórnych. U niektórych pacjentów nasilenie wykwitów skórnych zmniejsza się także po uzupełnieniu niedoborów aminokwasów i cynku.
Jeżeli w obrębie zmian skórnych doszło do nadkażeń, stosuje się miejscowe leki przeciwgrzybicze lub przeciwbakteryjne. W przypadku uporczywego świądu wdraża się leczenie przeciwhistaminowe.
Źródło: ncbi.nlm.nih.gov
Przeczytaj także: Jaki związek mają zmiany skórne na ciele z chorobą jelit lub nowotworem?
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