Pemphigus Paraneoplastik pada Timoma

Herley Windo Setiawan, Daniel Maranatha

= http://dx.doi.org/10.20473/jr.v4-I.1.2018.5-11
Abstract views = 182 times | views = 582 times

Abstract


Thymomas are mediastinal primary tumour of the mediastinum and associated with a variety of autoimmune disorders often linked to T-cell mediated autoimmunity. Paraneoplastic pemphigus (PNP) is an immunologically mediated skin disease characterized by epidermal blisters that may occur in association with thymic or non thymic neoplasms. Case:
21 years old man came to the hospital with vesicles and ulcer in oral cavity and extended to whole body since 3 weeks before. Patient was diagnosed with Pemphigus vulgaris and thymomas since 2 years. Patient was hospitalized for 3 times with same cases. Patient underwent chemotherapy Carboplatin-Etoposide 6 series and Radiotherapy 25 series and no response had been documented. Patient was suggested to undergo a resection of thymoma to prevent the exacerbation of pemphigus but he refused. The medication given was only aimed for reducing the exacerbation of Pemphigus. Discussion:
PNP is characterized by the production of autoantibodies against various target antigens, mainly plakin family proteins and desmogleins. PNP results from an antitumor immune response cross-reacting with the normal epithelial proteins and thereby inducing autoimmunity by molecular mimicry. PNP sera bind to homologous region within carboxyterminus of members of the plakin gene family. There are also involvement of dysregulation of T-cell development. Conclusion: Pemphigus is rarely associated with Thymomas, but the fact there is an association between Thymomas and pemphigus. According to this fact, every patients with pemphigus should be suspected with malignancies especially thymomas until it was ruled out.


Keywords


Thymomas, Pemphigus, PNP

Full Text:

PDF

References


Scorsetti M., Leo F., Trama A., D’Angelillo R., Serpico D., Macerelli M., Zucali P., et al. Thymoma and Thymic Carcinoma. Critical Reviews in Oncology/Hematology. 2016. 99: 332-350.

Marx A., Ströbel Ph., Zetti A., Chan JKC., Müller-Hermelink HK., Harris NL., et al. Thymomas. In: Pathology & Genetics. Tumours of Lung, Pleura, Thymus and Heart. Editor: Travis W.D., Brambilla E., Müller-Hermelink HK., and Harris CC. Publish by IARC Press. 2004: 152 – 153

Lee S.E., Kim S.C. Paraneoplastic Pemphigus. Dermatologica Sinica. 2010. 28: 1-14

Cho Y.T., Kao J.T., Chen H.J., Wang L.F., Chu C.Y. Paraneoplastic pemphigus: A retrospective case series in a referral center in northern Taiwan. Dermatologi Sinica. 2014. 32: 1-6

Yong A. A., Tey H. L. Paraneoplastic pemphigus. Australasian Journal of Dermatology. 2012. 54: 1-10

Edgin W A., Pratt T C., Grimwood R E. Pemphigus Vulgaris and Paraneoplastic Pemphigus. Oral Maxillofacial Surg Clin N Am. 2008. 20: 577-584

Saraya T., Fujiwara M., Shimura C., Goto H. Not paraneoplastic pemphigus but pemphigus vulgaris in a patient with thymoma. BMJ Case Rep. 2015.

Goldberg LJ., Nisar N. Paraneoplastic Pemphigus. The Medscape Drugs & Disease. 2016. Diunduh dari: http://emedicine. medscape.com/article/ 1064452 pada 30 Oktober 2016

Venugopal S S., Murell D F. Diagnosis and Clinical Features of Pemphigus Vulgaris. Dermatol Clin. 2011. 29: 373-380

Zeina B., Mansoor S., Sakka N. Pemphigus Vulgaris. The Medscape Drugs & Disease. 2016. Diunduh dari: http://emedicine.medscape. com/ article/1064187 pada 30 Oktober 2016

National Comprehensive Cancer Network. NCCN Guidelines Thymomas and Thymic Carcinomas. Version 2. NCCN. 2016

Evans K.J., Kline A.L., Miller Q. Thymomas. The Medscape Drugs & Disease. 2015. Diunduh dari: http://emedicine.medscape.com/ article/ 193809 pada 30 Oktober 2016

Yoshida M., Miyoshi T., Sakiyama S., Kondo K., Tangoku A. Pemphigus with thymoma improved by thymectomy: report of a case. Surg Today. 2013. 43: 806 – 808

Barbetakis N., Samanidis G., Paliouras D., Boukovinas I., Asteriou C., Stergiou E., et al. Paraneoplastic pemphigus regression after thymoma resection. World Journal of Surgical Oncology. 2008. 6: 83-86


Refbacks

  • There are currently no refbacks.


Copyright (c) 2019 Jurnal Respirasi

Creative Commons License
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.

View My Satats

Indexed by: