Atypical paraneoplastic pemphigus secondary to endometrial carcinoma with no mucosal involvement.
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.
Flinders Medical Centre, SA, Australia. nick@nick-kennedy.com
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Kennedy
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Dawe
D000230:Adenocarcinoma / Q000150:complications*; D000368:Aged; D001706:Biopsy; D016889:Endometrial Neoplasms / Q000150:complications*; D005260:Female; D006801:Humans; D010257:Paraneoplastic Syndromes / Q000209:etiology* / Q000473:pathology; D010392:Pemphigus / Q000209:etiology* / Q000473:pathology; D012867:Skin / Q000473:pathology