CASE REPORTS
JOURNAL ARTICLE
REVIEW
Add like
Add dislike
Add to saved papers

Clinical and immunopathological spectrum of paraneoplastic pemphigus.

BACKGROUND: Paraneoplastic pemphigus (PNP) is a rare life-threatening autoimmune bullous disorder. The clinical picture is often polymorphous and blisters may be absent. Autoantibodies are directed against several target antigens, including plakins and desmogleins. PNP has a high mortality rate that does not directly depend on the malignancy of the neoplasm. No generally accepted diagnostic criteria for PNP have been defined.

PATIENTS AND METHODS: On the basis of four selected PNP patients and the relevant literature, the spectrum of clinical and immunopathological findings as well as pathogenesis and treatment options of PNP are presented.

RESULTS: In addition to a neoplasm, severe stomatitis and the presence of autoantibodies against periplakin and envoplakin are characteristic for PNP. Based on the presented data, diagnostic criteria are proposed.

CONCLUSIONS: Knowledge of the polymorphous clinical picture and the complex autoantibody response is essential for an early diagnosis of PNP which has implications for both prognosis and rapid initiation of treatment.

Full text links

We have located links that may give you full text access.
Can't access the paper?
Try logging in through your university/institutional subscription. For a smoother one-click institutional access experience, please use our mobile app.

For the best experience, use the Read mobile app

Mobile app image

Get seemless 1-tap access through your institution/university

For the best experience, use the Read mobile app

All material on this website is protected by copyright, Copyright © 1994-2024 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

By using this service, you agree to our terms of use and privacy policy.

Your Privacy Choices Toggle icon

You can now claim free CME credits for this literature searchClaim now

Get seemless 1-tap access through your institution/university

For the best experience, use the Read mobile app