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CASE REPORT
Year : 2017  |  Volume : 8  |  Issue : 6  |  Page : 478-481

Paraneoplastic pemphigus with underlying retroperitoneal inflammatory myofibroblastic tumor: A case report and review of the literature


1 Skin Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran
2 Department of Pharmacology, Islamic Azad University, Science and Research Branch, Tehran, Iran

Correspondence Address:
Fahimeh Abdollahimajd
Skin Research Center, Shahid Beheshti University of Medical Sciences, Shohada-e-Tajrish Hospital, Shahrdari St, 1989934148, Tehran
Iran
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/idoj.IDOJ_17_17

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Inflammatory myofibroblastic tumor (IMT) is a peculiar low-grade neoplasm of spindle cell fibroblasts and myofibroblasts in an inflammatory background. The lung is the most common site of involvement. Here, we report a case of paraneoplastic pemphigus (PNP)-associated with an extensive retroperitoneal IMT. The patient had a favourable response following treatment with a low dose of systemic steroid, mycophenolate mofetil, and intravenous immunoglobulin (IVIG). He subsequently underwent surgery for resection of the tumor with nephrectomy and five courses of IVIG were administered after surgery due to a minor relapse. He was in remission in her last follow-up visit 16 months after surgery. The occurrence of PNP with IMT is notable; early detection and treatment are crucial for this tumor-associated autoimmune disease.


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