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Case Reports in Dermatological Medicine
Volume 2012 (2012), Article ID 207126, 6 pages
http://dx.doi.org/10.1155/2012/207126
Case Report

Paraneoplastic Pemphigus: A Paraneoplastic Autoimmune Multiorgan Syndrome or Autoimmune Multiorganopathy?

1Department of Dermatology, Venereology & Leprosy, Dr. R. P. Govt. Medical College, Kangra, Tanda, Himachal Pradesh 176001, India
2Department of Pathology, Dr. R. P. Govt. Medical College, Kangra, Tanda, Himachal Pradesh 176001, India
3Department of Radiotherapy & Oncology, Dr. R. P. Govt. Medical College, Kangra, Tanda, Himachal Pradesh 176001, India

Received 30 October 2012; Accepted 3 December 2012

Academic Editors: J. Y. Lee, N. Oiso, and R. Strohal

Copyright © 2012 Vikram K. Mahajan et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Paraneoplastic pemphigus (PNP), a clinically and immunopathologically distinct mucocutaneous blistering dermatosis, is a severe form of autoimmune multiorgan syndrome generally associated with poor therapeutic outcome and high mortality. This IgG-mediated disease is initiated by an obvious or occult lymphoproliferative disorder in most cases. Clinically severe mucositis, and polymorphic blistering skin eruptions, and histologically acantholysis, keratinocyte necrosis and interface dermatitis are its hallmark features. A 58-year-old female presented with recurrent, severe, recalcitrant stomatitis and widespread erosions/blistering lesions of one-year duration. Treatment with repeated courses of systemic corticosteroids at a peripheral center would provide temporary relief. She also had fever, productive cough, odynophagia and poor oral intake, herpes zoster ophthalmicus, pain in the abdomen, and watery diarrhea. An array of investigations revealed chronic lymphocytic leukemia (CLL), mediastinal and para-aortic lymphadenopathy, bronchiolitis obliterans, and vertebral osteoporosis/fractures. With the diagnosis of CLL-associated PNP she was managed with dexamethasone-cyclophosphamide pulse (DCP) therapy for 3 cycles initially, followed by COP regimen (cyclophosphamide, vincristine, and prednisolone) for 5 cycles. Remission is being maintained with chlorambucil and prednisolone pulse therapy once in 3 weeks with complete resolution of skin lesions and adequate control of CLL.