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Paraneoplastic pemphigus

Minhee Kim, Luca Borradori, Dédée F Murrell
Elderly patients are more susceptible to the development of autoimmune blistering disorders such as bullous pemphigoid, mucous membrane pemphigoid, epidermolysis bullosa acquisita, and paraneoplastic pemphigus. This article focuses on the clinical aspects of the aforementioned autoimmune blistering diseases and highlights the important factors involved in treating elderly patients. It is essential for clinicians to offer individualized treatment plans for these patients to optimize outcomes, as elderly patients often have multiple co-morbidities, polypharmacy, and suboptimal socioeconomic status that can adversely influence adequate compliance...
October 13, 2016: Drugs & Aging
Marta Wieczorek, Annette Czernik
Paraneoplastic pemphigus (PNP) is a fatal autoimmune blistering disease associated with an underlying malignancy. It is a newly recognized blistering disease, which was first recognized in 1990 by Dr Anhalt who described an atypical pemphigus with associated neoplasia. In 2001, Nguyen proposed the term paraneoplastic autoimmune multiorgan syndrome because of the recognition that the condition affects multiple organ systems. PNP presents most frequently between 45 and 70 years old, but it also occurs in children and adolescents...
2016: Clinical, Cosmetic and Investigational Dermatology
Sun-Young Lee, Jong-Hyun Kim, Dong-Hyu Cho
Paraneoplastic autoimmune multiorgan syndrome (PAMS), also known as paraneoplasic pemphigus, involves the skin, internal organs and mucosa. PAMS-associated mortality may occur as a result of autoantibody formation against internal tumors and their infiltration into organs other than the skin lesions that characterize PAMS. The most common symptoms of PAMS include pain associated with continuous oral ulceration and resistance to pharmacological treatment. The present study reports the case of a 42-year-old female patient who was admitted with an 8-month history of erosive skin lesions within the trunk region, oral mucosa and vaginal mucosa...
September 2016: Experimental and Therapeutic Medicine
Raffaele Piscopo, Mary Romano, Alessandra Di Maria, Riccardo Vinciguerra, Paolo Vinciguerra
INTRODUCTION: Paraneoplastic clinical signs are characterized by a large and heterogeneous variety of manifestations due to several possible underlying neoplasms. Paraneoplastic pemphigus (PNP) is a particular paraneoplastic variety that usually primarily affects the dermic and/or oral mucosa and is characterized by a high rate of mortality (90%). Therefore, it is important to recognize its possible signs early. This report describes a case of ocular paraneoplastic pemphigus (PNP) presenting with recalcitrant eyelid ulceration and hyperemic conjunctivitis caused by an undiagnosed prostate cancer...
August 18, 2016: Ocular Immunology and Inflammation
Lifang Wang, Hui Deng, Mei Mao
Paraneoplastic pemphigus (PNP) is an autoimmune blistering disease associated with neoplasms. The disease is most commonly of lymphoproliferative origin and presents high mortality. We describe a patient with PNP and myasthenia gravis associated with inflammatory pseudotumor-like follicular dendritic cell sarcoma, as well as the response to rituximab.
August 2016: Clinical Case Reports
Xue Wang, Tiancheng Chen, Junyu Zhao, Yang Peng, Xixue Chen, Ping Tu, Xuejun Zhu, Zhi Liu, Mingyue Wang
BACKGROUND: Autoantibodies against N-terminal domains and linker subdomains of envoplakin (EVPL) and periplakin (PPL) were frequently detected in sera of paraneoplastic pemphigus(PNP) patients. OBJECTIVES: To further investigate finer epitopes of EVPL and PPL, and evaluate their associations with clinical aspects of PNP. METHODS: We produced 12 overlapping truncated fragments of these regions in Escherichia coli, and measured their reactivities to sera of 65 PNP patients and 50 healthy volunteers by enzyme-linked immunosorbent assays (ELISA)...
June 23, 2016: Journal of Dermatological Science
Haijun Gong, Shiyou Zhou, Yuxin Hu, Yuqin Lan, Hong Zeng, Liangchun Wang, Qingyu Liu, Mei Wang
BACKGROUND: The ocular presentation of Castleman's disease (CD)-associated paraneoplastic pemphigus (PNP) has rarely been reported. In this report, we describe a young patient with CD-associated PNP who had recurrent corneal ulceration in addition to cicatrizing conjunctivitis. CASE PRESENTATION: We describe a case of 23-year-old male with mucocutaneous erosion and conjunctival injection and erosion who was found to have PNP. Pelvic hyaline-vascular CD was detected and completely excised...
2016: BMC Ophthalmology
Salahuddin Siddiqui, Mohammad Bilal, Zachary Otaibi, Farshaad Bilimoria, Nihar Patel, James Rossetti
Skin lesions are frequently encountered in clinical practice which can be a presentation of systemic diseases not excluding an occult malignancy. Commonly reported paraneoplastic dermatologic manifestations include acanthosis nigricans, dermatomyositis, erythroderma, hypertrophic osteoarthropathy, Sweet syndrome, and paraneoplastic pemphigus (PNP). PNP is a rare autoimmune mucocutaneous disease characterized by severe stomatitis, polymorphic skin eruptions, and associated underlying neoplasms most commonly non-Hodgkin's lymphoma, chronic lymphocytic leukemia, and Castleman disease...
June 23, 2016: Hematology/oncology and Stem Cell Therapy
Yan-Zhao Wang, Zhao-Ya Gao, Fu-Ming Lei, Jin-Xia Zhang, Jin Gu
No abstract text is available yet for this article.
June 20, 2016: Chinese Medical Journal
Zhipeng Zhang, Maosong Zhou, Jin Guo, Tiecheng Feng, Xinying Li, Huan Chen, Jindong Li
Paraneoplastic pemphigus is a rare autoimmune bullous dermatosis, which is caused by potential neoplasm, especially the Castleman's disease. Castleman's disease associated with paraneoplastic pemphigus is misdiagnosed frequently and easily in clinical practices. Furthermore, it is reported that the mortality rate for this disease is very high. Bronchiolitis obliterans is the most common complication and the most important cause of death. There was a female patient presenting recalcitrant mucocutaneous erosions, ulcers and scattered erythemas in the body...
May 2016: Zhong Nan da Xue Xue Bao. Yi Xue Ban, Journal of Central South University. Medical Sciences
Aparna Mullangath Prakasan, Anne Jennifer Prabhu, Kanmani Velarasan, Selvamani Backianathan, Thomas Samuel Ram
Paraneoplastic Pemphigus (PNP) is an autoimmune bullous disease characterized by severe stomatitis, polymorphous skin eruptions, and underlying neoplasms. Diagnosis of cutaneous paraneoplastic disorders requires high index of suspicion. We describe a patient with PNP associated with follicular dendritic cell (FDC) tumor in the mediastinum, a rare neoplasm originating from follicular dendritic cells. Its management requires identification of underlying malignancy and treatment of the same. Our patient showed remission of PNP upon excision of the tumor and remained disease-free for 8 years...
2016: Case Reports in Dermatological Medicine
Evelyne Halpert, Juan Luis Figueroa, Armando Rojas, Clara Ines Ortiz, Daniela Chaparro, Marcela Galindo, Juan Javier Lammoglia, Carolina Rumie, Carlos Olmos
No abstract text is available yet for this article.
January 2016: JAAD Case Reports
Santosh Kumar, Kshitij Bishnoi, Pragatheeswarane Murugavaithianathan, Vikas Kumar Panwar
Castleman's disease is a rare, benign lymphoproliferative disorder of unknown origin. Paraneoplastic pemphigus is a common association which presents as oral mucosal ulcerations. Abdominal and retroperitoneal Castleman's disease present either as a localized disease or as a systemic disease. We hereby present a 15-year-old male patient with oral mucosal lesions with localized vague right lower abdominal mass who was diagnosed to have Castleman's disease with paraneoplastic pemphigus which was surgically excised...
2016: Journal of Clinical Imaging Science
Yohei Otsuka, Takashi Ueno, Aya Yamase, Michiko Ito, Shinichi Osada, Seiji Kawana, Yoko Funasaka, Kwesi Teye, Norito Ishii, Takashi Hashimoto, Hidehisa Saeki
A 63-year-old Japanese man with non-Hodgkin B-cell lymphoma presented with erythematous skin lesions on his entire body, with oral, ocular and anal mucosal lesions. The patient was diagnosed with paraneoplastic pemphigus. Immunofluorescence showed both immunoglobulin (Ig)G and IgA antibodies to keratinocyte cell surfaces. Various immunoblot and enzyme-linked immunosorbent assays showed both IgG and IgA antibodies to various autoantigens, including desmogleins, desmocollins, envoplakin, periplakin and bullous pemphigoid antigens...
August 2016: Journal of Dermatology
Karen C Broussard, Theresa G Leung, Ahmadreza Moradi, Jennifer E Thorne, Jo-David Fine
Autoimmune blistering diseases are a heterogeneous group of disorders that mostly affect the skin and mucous membranes. Occasionally, other organ systems may be involved, depending on the unique pathophysiology of each disease. Cicatricial pemphigoid, pemphigus vulgaris, and paraneoplastic pemphigus are distinct entities, but all have the potential to have cutaneous and ocular involvement. Awareness and early recognition of ocular involvement in these diseases is important given the increased risk for vision loss and blindness with delay in management...
March 2016: Clinics in Dermatology
Norwani Basir, Pemasari Upali Telisinghe, Vui Heng Chong
Paraneoplastic pemphigus is a relatively rare but significant acquired autoimmune mucocutaneous disorder that is characterised by diffuse erythema, painful blistering and sores of the skin and mucus membranes. The underlying pathogenesis is believed to be triggered by altered immune system in response to underlying neoplasm. The manifestations can predate, occur at the same time or after the diagnosis of cancer. Associations with gastric cancer have only been reported twice. A 78-year-old lady presented with a month's history of extensive skin lesions that started off as bullous lesions and biopsy revealed bullous pemphigus...
December 2015: Indian Journal of Surgery
Atsunari Tsuchisaka, Sanae Numata, Kwesi Teye, Yohei Natsuaki, Tamihiro Kawakami, Yoshito Takeda, Wenqing Wang, Kazushi Ishikawa, Mizuki Goto, Hiroshi Koga, Ryosuke Sogame, Norito Ishii, Shinzo Takamori, Tomoaki Hoshino, Oliver Brandt, Hendri H Pas, Sakuhei Fujiwara, Takashi Hashimoto
All plakin family proteins are known to be autoantigens in paraneoplastic pemphigus (PNP). In this study, we first examined whether PNP sera also react with epiplakin, another plakin protein, by various immunological methods using 48 Japanese PNP sera. Immunofluorescence confirmed that cultured keratinocytes expressed epiplakin. Epiplakin was detected by 72.9% of PNP sera by immunoprecipitation-immunoblotting with KU-8 cell extract, but not by immunoblotting of either normal human epidermal extract or KU-8 cell extract...
February 2016: Journal of Investigative Dermatology
E Sprecher
No abstract text is available yet for this article.
December 2015: British Journal of Dermatology
Kalyani Marathe, Jun Lu, Kimberly D Morel
Bullous diseases may be rare; however, this does not preclude the clinician from being familiar with their manifestations and treatment. After ruling out infection, genetically inherited blistering diseases are more likely to be the cause of blistering or erosions in the neonatal period, whereas immunobullous diseases are more common in adults. Published literature on immunobullous disorders reflects information gleaned from case reports and open-label case series; prospective studies and evidence-based treatments are limited...
November 2015: Clinics in Dermatology
Chika Namba, Mikiko Tohyama, Yasushi Hanakawa, Masamoto Murakami, Yuji Shirakata, Takuya Matsumoto, Koichiro Suemori, Norito Ishii, Takashi Hashimoto, Koji Sayama
Paraneoplastic pemphigus (PNP) is an autoimmune blistering disease that presents as severe mucosal erosions and variable cutaneous lesions and is primarily associated with hematologically malignant or benign diseases. A 59-year-old Japanese woman presented with oral, ocular and vaginal mucosal erosions and erythema as well as blistering on her trunk and limbs. She developed bronchiolitis obliterans; lymphadenopathy in the cervical, subclavian, para-aortic and intraperitoneal regions; and splenomegaly. PNP with B-cell lymphoma was diagnosed...
April 2016: Journal of Dermatology
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