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Paraneoplastic Pemphigus : A Paraneoplastic Autoimmune Multiorgan Syndrome or Autoimmune Multiorganopathy?
Joint Authors
Sharma, Vikas
Negi, Muninder
Mahajan, Vikram K.
Mehta, Karaninder S.
Prabha, Neel
Abhinav, C.
Chauhan, Pushpinder S.
Sharma, Saurabh
Sharma, Anju Lath
Khatri, Gayatri
Source
Case Reports in Dermatological Medicine
Issue
Vol. 2012, Issue 2012 (31 Dec. 2012), pp.1-6, 6 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2012-12-19
Country of Publication
Egypt
No. of Pages
6
Main Subjects
Abstract EN
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.
American Psychological Association (APA)
Mahajan, Vikram K.& Sharma, Vikas& Chauhan, Pushpinder S.& Mehta, Karaninder S.& Sharma, Anju Lath& Abhinav, C.…[et al.]. 2012. Paraneoplastic Pemphigus : A Paraneoplastic Autoimmune Multiorgan Syndrome or Autoimmune Multiorganopathy?. Case Reports in Dermatological Medicine،Vol. 2012, no. 2012, pp.1-6.
https://search.emarefa.net/detail/BIM-454519
Modern Language Association (MLA)
Mahajan, Vikram K.…[et al.]. Paraneoplastic Pemphigus : A Paraneoplastic Autoimmune Multiorgan Syndrome or Autoimmune Multiorganopathy?. Case Reports in Dermatological Medicine No. 2012 (2012), pp.1-6.
https://search.emarefa.net/detail/BIM-454519
American Medical Association (AMA)
Mahajan, Vikram K.& Sharma, Vikas& Chauhan, Pushpinder S.& Mehta, Karaninder S.& Sharma, Anju Lath& Abhinav, C.…[et al.]. Paraneoplastic Pemphigus : A Paraneoplastic Autoimmune Multiorgan Syndrome or Autoimmune Multiorganopathy?. Case Reports in Dermatological Medicine. 2012. Vol. 2012, no. 2012, pp.1-6.
https://search.emarefa.net/detail/BIM-454519
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-454519