Paraneoplastic pemphigus associated with inflammatory myofibroblastic tumour of the mediastinum: A favourable response to treatment and review of the literature.
Australas J Dermatol
; 56(2): 120-3, 2015 May.
Article
em En
| MEDLINE
| ID: mdl-25369175
ABSTRACT
Paraneoplastic pemphigus (PNP) is an autoimmune blistering disorder that occurs in association with an underlying neoplasm. Inflammatory myofibroblastic tumour (IMT) is a rare low-grade sarcoma of fibroblasts and myofibroblasts associated with inflammatory cells, most commonly occurring in the lung. In this study, a case of PNP associated with IMT of the mediastinum is reported. The patient had a favourable outcome following surgical resection and treatment with a systemic steroid, azathioprine, and i.v. immunoglobulin. The occurrence of PNP with sarcomas, specifically IMT, is noteworthy although it is not well studied in the existing literature.
Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Síndromes Paraneoplásicas
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Pênfigo
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Neoplasias do Mediastino
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Neoplasias de Tecido Muscular
Tipo de estudo:
Etiology_studies
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Risk_factors_studies
Limite:
Adult
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Female
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Humans
Idioma:
En
Ano de publicação:
2015
Tipo de documento:
Article