Acta Medica Iranica 2017. 55(5):340-343.

A Case Report of Paraneoplastic Pemphigus Associated With Retroperitoneal Inflammatory Myofibroblastic Tumor
Kamran Balighi, Arghavan Azizpour, Ali Sadeghinia, Vahide Saeidi

Abstract


Paraneoplastic pemphigus (PNP) is an autoimmune bullous disease associated with underlying neoplasms, both malignant and benign. The most constant clinical presentation of PNP is the presence of intractable stomatitis. Herein we present a 25-year-old male with a 3-month history of refractory stomatitis especially involving the lips and widespread vesiculobullous eruption on his trunk and extremities. The diagnosis of PNP was confirmed based on histological and serological results. Investigation for the underlying neoplasm revealed a retroperitoneal tumorous mass which was biopsied and diagnosed as the inflammatory myofibroblastic tumor (IMT). The tumor was surgically excised, and different treatment regimens were used to treat the mucocutaneous lesions. Skin lesions responded favorably to treatment, but oral stomatitis still persists which is the case in most PNP patients. This combination of PNP and IMT has rarely been reported in the literature. Treatment started with corticosteroid and rituximab then tumor excised.


Keywords


Paraneoplastic pemphigus; Myofibroblastic tumor; Stomatitis

Full Text:

PDF

References


Anhalt GJ, Kim SC, Stanley JR et al. Paraneoplastic Pemphigus: an autoimmune

mucocutaneous disease associated with neoplasia. N Engl Med 1990; 323:1729-35.

Nguyen VT, Ndoye A, Bassler KD et al. Classification, clinical manifestations, and

immunological mechanisms of the epithelial variant of paraneoplastic autoimmune multiorgan

syndrome: a reappraisal of paraneoplastic pemphigus. Arch Dermatol 2001; 137: 193-206.

Lee SE, Kim SC. Paraneoplastic pemphigus. Dermatol Sin 2010;2013:1-14.

Chen CH,Huang WC,Liu HC et al.Surgical outcome of inflammatory pseudotumor in the

lung.Thorac. Cardiovassc. Surg.2008;56:214-6.

Anhalt GJ. Paraneoplastic pemphigus. J Investing Dermatol Symp Proc 2004; 9: 29-33.

Camisa C, Heim TN. Paraneoplastic pemphigus is a distinct neoplasia-induced autoimmune

disease. Arch Dermatol. 1993; 129: 883-6.

Ghandi N,Ghanadan A, Azizian MRet al.Paraneoplastic pemphigus associated with

inflammatory myofibroblastic tumours of the mediastinum: A favarable response to treatment

review of the litterer. Australas J Dermatol. Willey-blackwell 2014.

Narla LD, Newman B, Spottswood SS, Narla S, Kolli R. Inflammatory pseudotumor.

Radiographics. 2003; 23: 719-729.

Lu CH, Huang HY, Chen HK, Chuang JH, Ng SH, Ko SF. Huge pelvi-abdominal malignant

inflammatory myofibroblastic tumor with rapid recurrence in a 14-year-old boy. World J

Gastroenterol. 2010; 12: 2698-2701.

Fletcher CD, Bridge JA, Hogendoorn PC. WHO Classification of Tumors of Soft Tissue and

Bone. Lyon: International Agency for Research on Cancer;2013.p.83.

Pavithran K, Manoj P, Vidhyadharan G, Shanmughasundaram P. Inflammatory Myofibroblastic

Tumor of the Lung: Unusual Imaging Findings. World J Nucl Med 2013; 12: 126-8.


Refbacks

  • There are currently no refbacks.


Creative Commons Attribution-NonCommercial 3.0

This work is licensed under a Creative Commons Attribution-NonCommercial 3.0 Unported License which allows users to read, copy, distribute and make derivative works for non-commercial purposes from the material, as long as the author of the original work is cited properly.