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Paraneoplastic pemphigus

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https://www.readbyqxmd.com/read/28492232/pemphigus
#1
REVIEW
Michael Kasperkiewicz, Christoph T Ellebrecht, Hayato Takahashi, Jun Yamagami, Detlef Zillikens, Aimee S Payne, Masayuki Amagai
Pemphigus is a group of IgG-mediated autoimmune diseases of stratified squamous epithelia, such as the skin and oral mucosa, in which acantholysis (the loss of cell adhesion) causes blisters and erosions. Pemphigus has three major subtypes: pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus. IgG autoantibodies are characteristically raised against desmoglein 1 and desmoglein 3, which are cell-cell adhesion molecules found in desmosomes. The sites of blister formation can be physiologically explained by the anti-desmoglein autoantibody profile and tissue-specific expression pattern of desmoglein isoforms...
May 11, 2017: Nature Reviews. Disease Primers
https://www.readbyqxmd.com/read/28401573/is-tocilizumab-a-potential-therapeutic-option-for-refractory-unicentric-castleman-disease
#2
Muhammad Bilal Abid, Rachel Peck, Muhammad Abbas Abid, Wesam Al-Sakkaf, Yuening Zhang, Giles S Dunnill, Konrad Staines, Iara-Maria Sequeiros, Lisa Lowry
Castleman disease is a rare lymphoproliferative disorder with 2 distinctly defined clinical forms. While multicentric Castleman disease (UCD) poses a potential therapeutic challenge, unicentric variant has historically been considered curable with surgical resection. Hence, little is known to guide management of patients with UCD, refractory to surgical resection and combination chemotherapy. We present a case of a patient, negative for HIV and HHV-8, who had an unsuccessful surgical intervention and no response to radiotherapy and chemotherapy...
April 11, 2017: Hematological Oncology
https://www.readbyqxmd.com/read/28394553/drug-induced-oral-ulcerations-case-report
#3
Vanja Vučičević Boras, Snježana Židovec-Lepej, Branka Marinović, Sven Seiwerth, Ivana Škrinjar, Dražen Pulanić, Danica Vidović Juras
A 70-year-old patient was admitted to the Department of Oral Medicine for multiple oral ulcerations on the left buccal mucosa, around 0.5 cm in diameter, as well as on the gingiva. Otherwise, the patient suffered from chronic lymphocytic leukemia, hypogammaglobulinemia, chronic renal insufficiency, with complete afunction of the right kidney, asthma, hypertension, gastritis and prostate hyperplasia. Differential diagnosis of oral ulcerations included drug induced oral ulcerations, paraneoplastic pemphigus, viral ulcerations (cytomegalovirus, herpes simplex viruses), fungal ulcerations (candidiasis, aspergillosis, histoplasmosis, cryptococcosis) and bacterial ulcerations, as well as neutropenic ulcers...
June 2016: Acta Clinica Croatica
https://www.readbyqxmd.com/read/28392652/paraneoplastic-pemphigus-associated-with-a-malignant-thymoma-a-case-of-persistent-and-refractory-oral-ulcerations-following-thymectomy
#4
Jung Min Lim, Sang Eun Lee, Jimyung Seo, Do Young Kim, Takashi Hashimoto, Soo-Chan Kim
Paraneoplastic pemphigus is a rare, life-threatening autoimmune mucocutaneous blistering disease associated with underlying neoplasia, commonly lymphoproliferative tumors. Herein we report a case of paraneoplastic pemphigus with a unique autoantibody profile associated with a malignant thymoma. A 56-year-old female patient presented with relapsing oral ulcerations accompanied by erythematous papules and patches on her extremities for 2 months. Skin and mucosal biopsies identified interface dermatitis with lichenoid lymphocytic infiltration in the upper dermis...
April 2017: Annals of Dermatology
https://www.readbyqxmd.com/read/28367488/atypical-laboratory-presentation-of-paraneoplastic-pemphigus-associated-with-castleman-disease
#5
Sarah Lonowski, Hayley Goldbach, Vanessa Holland
No abstract text is available yet for this article.
March 2017: JAAD Case Reports
https://www.readbyqxmd.com/read/28361863/trichilemmal-cystis-in-metastatic-melanoma-a-case-report
#6
I Savarese, M Grazzini, A Gori, A D'Errico, L Doni, F Scarfì, P Covarelli, F Di Costanzo, V De Giorgi
The malignant melanoma is a neoplasm associated with a wide variety of cutaneous paraneoplastic syndromes, as dermatomyositis, systemic sclerosis, paraneoplastic pemphigus. We describe a case of four multiple trichilemmal cystis arising on frontal region in the same patient with brain metastasis and unknown primary melanoma and discuss their relationship.
March 2017: Experimental Oncology
https://www.readbyqxmd.com/read/28295171/successful-use-of-bruton-s-kinase-inhibitor-ibrutinib-to-control-paraneoplastic-pemphigus-in-a-patient-with-paraneoplastic-autoimmune-multiorgan-syndrome-and-chronic-lymphocytic-leukaemia
#7
Andrew Lee, Suneet Sandhu, Louise Imlay-Gillespie, Stephen Mulligan, Stephen Shumack
We present the case of a 51-year-old man who developed paraneoplastic pemphigus (PNP) in the context of chronic lymphocytic leukemia (CLL). His CLL was successfully controlled with ibrutinib. Concurrently, there was significant improvement of his PNP, suggesting that ibrutinib may be a very useful addition to the treatment options in this potentially life-threatening autoimmune disorder.
March 13, 2017: Australasian Journal of Dermatology
https://www.readbyqxmd.com/read/28225504/paraneoplastic-pemphigus-associated-with-chronic-lymphocytic-leukemia-a-case-report
#8
Qu Jiang, Bin Hong Zhang
RATIONALE: Paraneoplastic pemphigus (PNP) is an autoimmune syndrome associated with neoplasms. The treatment approach principally includes suppressing the immunity, but its therapeutic effect is not satisfying. PATIENT CONCERNS: We report a case of paraneoplastic pemphigus linked to chronic lymphocytic leukemia in a 63-year-old man. DIAGNOSES: At first, the patient was diagnosed with pityriasis rose caused by a viral infection. Biopsies for histology and immunofluorescence showed PNP, was treated with immunosuppressive and antiinfective therapy...
February 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27914107/non-paraneoplastic-autoimmune-subepidermal-bullous-disease-associated-with-fatal-bronchiolitis-obliterans
#9
Mari Orime, Katsuhiro Tomiyama, Hideki Hashidate, Satoru Yoshida, Satoshi Hokari, Akiko Tsuda, Hisashi Yokoyama, Jun-Ichi Narita, Youhei Uchida, Takuro Kanekura, Riichiro Abe, Norito Ishii, Takashi Hashimoto, Kazuhiro Kawai
Bronchiolitis obliterans is a small-airway obstructive lung disease for which immunologically mediated pathogenesis is supposed. Frequent association of bronchiolitis obliterans with paraneoplastic pemphigus is well known, but its association with other autoimmune bullous diseases has not been reported except for a case of anti-laminin-332-type mucous membrane pemphigoid in a patient with chronic graft-versus-host disease. We report a case of non-paraneoplastic autoimmune subepidermal bullous disease associated with fatal bronchiolitis obliterans in a patient without transplantation...
April 2017: Journal of Dermatology
https://www.readbyqxmd.com/read/27902996/pemphigus-pathogenesis-to-treatment
#10
REVIEW
Christopher DiMarco
Pemphigus vulgaris (PV), pemphigus foliaceus (PF), and paraneoplastic pemphigus (PNP) are a group of rare and fatal blistering diseases involving autoantibodies that target desmosomal proteins. The pathogenesis of pemphigus involves the production of activated B-cells and IgG with stimulation by IL-4 by T-helper 2 cells. Clinically these diseases present most often with epidermal erosions of the mucosae and skin caused by rapid rupturing of flaccid bullae. These lesions correlate histologically with splits forming in the epidermis, leaving a blister roof composed of a few cell layers...
December 1, 2016: Rhode Island Medical Journal
https://www.readbyqxmd.com/read/27878477/paraneoplastic-pemphigus-and-autoimmune-blistering-diseases-associated-with-neoplasm-characteristics-diagnosis-associated-neoplasms-proposed-pathogenesis-treatment
#11
REVIEW
Saritha Kartan, Vivian Y Shi, Ashley K Clark, Lawrence S Chan
Autoimmune paraneoplastic and neoplasm-associated skin syndromes are characterized by autoimmune-mediated cutaneous lesions in the presence of a neoplasm. The identification of these syndromes provides information about the underlying tumor, systemic symptoms, and debilitating complications. The recognition of these syndromes is particularly helpful in cases of skin lesions presenting as the first sign of the malignancy, and the underlying malignancy can be treated in a timely manner. Autoimmune paraneoplastic and neoplasm-associated bullous skin syndromes are characterized by blister formation due to an autoimmune response to components of the epidermis or basement membrane in the context of a neoplasm...
February 2017: American Journal of Clinical Dermatology
https://www.readbyqxmd.com/read/27812271/peri-anal-paraneoplastic-pemphigus-heralding-the-relapse-of-follicular-lymphoma-and-its-successful-management-by-rituximab-a-short-correspondence
#12
Ankur Jain, Gaurav Prakash, Ram V Nampoothiri, Dipankar De, Amanjit Bal, Alka Khadwal, Deepesh Lad, Pankaj Malhotra, Subhash Varma
No abstract text is available yet for this article.
December 2016: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/27738963/autoimmune-blistering-diseases-in-the-elderly-clinical-presentations-and-management
#13
REVIEW
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 2016: Drugs & Aging
https://www.readbyqxmd.com/read/27729809/paraneoplastic-pemphigus-a-short-review
#14
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
https://www.readbyqxmd.com/read/27602070/high-dose-rate-intraluminal-brachytherapy-for-paraneoplastic-autoimmune-multiorgan-syndrome
#15
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
https://www.readbyqxmd.com/read/27537424/ocular-onset-of-paraneoplastic-pemphigus-presenting-as-hyperemic-conjunctivitis-and-massive-bilateral-eyelid-ulceration-a-case-report-and-literature-review
#16
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
https://www.readbyqxmd.com/read/27525088/paraneoplastic-pemphigus-and-myasthenia-gravis-associated-with-inflammatory-pseudotumor-like-follicular-dendritic-cell-sarcoma-response-to-rituximab
#17
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
https://www.readbyqxmd.com/read/27427435/extremities-of-the-n-terminus-of-envoplakin-and-c-terminus-of-its-linker-subdomain-are-major-epitopes-of-paraneoplastic-pemphigus
#18
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)...
October 2016: Journal of Dermatological Science
https://www.readbyqxmd.com/read/27406120/recurrent-corneal-melting-in-the-paraneoplastic-pemphigus-associated-with-castleman-s-disease
#19
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...
July 12, 2016: BMC Ophthalmology
https://www.readbyqxmd.com/read/27352260/paraneoplastic-pemphigus-as-a-presentation-of-acute-myeloid-leukemia-early-diagnosis-and-remission
#20
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
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