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Zmiany skórne w przebiegu pęcherzycy paraneoplastycznej

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Pęcherzyca paraneoplastyczna to schorzenie autoimmunologiczne rozwijające się w wyniku reakcji organizmu na nowotwór. W przebiegu choroby na skórze i błonach śluzowych rozwijają się bolesne i trudno gojące się nadżerki. Jak przebiega leczenie pęcherzycy paraneoplastycznej?

Pęcherzyca paraneoplastyczna to schorzenie towarzyszące chorobom nowotworowym, szczególnie nowotworom hematologicznym (chłoniak nieziarniczy, przewlekła białaczka limfocytarna, grasiczak). Jest związana z nieprawidłową odpowiedzią immunologiczną organizmu na toczący się proces nowotworowy, co prowadzi do powstania przeciwciał reagujących z własnymi tkankami organizmu.

Ze względu na to, że oprócz objawów śluzówkowo-skórnych choroba może dawać objawy ze strony płuc i innych narządów, alternatywnie określa się ją jako autoimmunologiczny paranowotworowy zespół wielonarządowy (ang. paraneoplastic autoimmune multi-organ syndrome, PAMS).

Obraz kliniczny pęcherzycy paraneoplastycznej

W przebiegu pęcherzycy paraneoplastycznej rozwijają się polimorficzne zmiany skórne, które mogą przypominać inne jednostki chorobowe. Do częstych objawów skórnych należą ogniska pęcherzowe oraz rumieniowo-złuszczające. W części przypadków obserwuje się również wykwity przypominające w obrazie klinicznym objawy rumienia wielopostaciowego lub liszaja płaskiego.

W obrębie błon śluzowych jamy ustnej, języka, podniebienia i dziąseł pojawiają się krwawiące nadżerki i owrzodzenia, z którymi wiążą się silne dolegliwości bólowe. Zmiany chorobowe mogą objąć również gardło i błonę śluzową nosa oraz czerwień wargową (obraz kliniczny jak w chorobie Stevensa-Johnsona). Zmiany wykazują duże trudności w gojeniu. Proces chorobowy może objąć również błony śluzowe narządów wewnętrznych, w tym przewodu pokarmowego, narządów płciowych oraz układu oddechowego (zarostowe zapalenie oskrzelików).

Pęcherzyca paraneoplastyczna może również powodować powikłania okulistyczne. Zmiany w obrębie spojówki i rogówki mogą stać się przyczyną trwałego uszkodzenia wzroku lub ślepoty.

Leczenie pęcherzycy paraneoplastycznej

W części przypadków wystąpienie objawów pęcherzycy paraneoplastycznej może wyprzedzić diagnozę towarzyszącego nowotworu. Diagnozę ustala się na podstawie badania histopatologicznego oraz oznaczenia swoistych autoprzeciwciał.

W terapii pęcherzycy paraneoplastycznej największe znaczenie ma leczenie choroby podstawowej, ponieważ pozwala to na zmniejszenie liczby produkowanych przeciwciał, a w konsekwencji złagodzenie objawów pęcherzycy. W leczeniu objawowym stosuje się leki sterydowe oraz immunosupresyjne. W przypadku nieskuteczności farmakoterapii wykorzystywana jest plazmafereza.


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