Multidisciplinary Clinical Management of Paraneoplastic Pemphigus – A Case Report

Paraneoplastic pemphigus is a rare immunobullous disorder commonly associated with lymphoproliferative neoplasms and less commonly in carcinomas with a poor prognosis. The neoplasms produce autoantibodies that react with members of plakin family to produce a suprabasilar split and varied clinical pr...

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Bibliographic Details
Published in:Journal of clinical and diagnostic research 2017-10, Vol.11 (10), p.ZD01-ZD03
Main Authors: Jayachandran, Sadaksharam, Preethi, M
Format: Article
Language:English
Subjects:
Immunobullous disorders
Immunomodulators
Lymphoproliferative neoplasms
Squamous cell carcinoma
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Summary:Paraneoplastic pemphigus is a rare immunobullous disorder commonly associated with lymphoproliferative neoplasms and less commonly in carcinomas with a poor prognosis. The neoplasms produce autoantibodies that react with members of plakin family to produce a suprabasilar split and varied clinical presentations in the skin and mucous membrane. Oral erosions are the first manifestation most resistant to treatment and persist even after the underlying tumour has been resected or treated. We report here a rare case of paraneoplastic pemphigus with an underlying squamous cell carcinoma of the oesophagus that constitutes only 8% of the neoplasms associated with this condition. It was successfully managed by a multidisciplinary approach involving the Departments of Dermatology, Surgical Oncology and Oral Medicine. The oral erosions were resistant to treatment however, a patient-tailored treatment was advocated using professionally administered debridement with hydrogen peroxide diluted with hypotonic saline, high potency topical steroids, analgesic oral rinses and topical anaesthetics with systemically administered immunomodulators. Hence, management of such cases exhibiting systemic and oral manifestations require a multidisciplinary team approach to improve the quality of life of the patients.
ISSN:2249-782X
0973-709X
2249-782X
DOI:10.7860/JCDR/2017/29160.10708