Atypical paraneoplastic pemphigus secondary to endometrial carcinoma with no mucosal involvement

Authors: Kennedy, N. A.; Dawe, S.

Source: Clinical & Experimental Dermatology, Volume 34, Number 5, July 2009 , pp. e130-e133(4)

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Abstract:

Summary

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.

Document Type: Research article

DOI: http://dx.doi.org/10.1111/j.1365-2230.2008.03150.x

Affiliations: 1: Department of Dermatology, Hemel Hempstead General Hospital, Hemel Hempstead, Hertfordshire, UK

Publication date: 2009-07-01