Paraneoplastic pemphigus (PNP) is a mucocutaneous immunobullous disease associated with several types of internal malignancy. We report a case of a 78-year-old woman presenting with an atypical form of PNP associated with a recurrence of endometrial cancer. There was no involvement of the mucous membranes. Although the macroscopic and histological appearances were typical for IgA pemphigus or Sneddon-Wilkinson disease, direct immunofluorescence was positive for intercellular IgG and C3 staining. Circulating antibodies were detected to periplakin and envoplakin, but not to desmogleins 1 or 3. This case therefore meets the modified criteria for PNP. A response was seen to a combination of topical and systemic corticosteroids and acitretin, although with cyclical recurrences. These ceased after resection of her recurrent endometrial carcinoma.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.