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Jiemei Cen, Wen Zeng, Mianluan Pan, Ye Qiu, Jianquan Zhang
Castleman disease (CD) is clinically divided into unicentric CD (UCD) and multicentric CD (MCD). Hyaline-vascular variant (HV) is the most common pathological type of UCD, while the plasma cell type (PC) is the most common type of MCD and thus, hyaline-vascular variant multicentric CD (HV-MCD) is a rare type of CD. In addition, its etiology has remained elusive. The present study retrospectively analyzed the medical records of 3 patients diagnosed as HV-MCD admitted to The First Affiliated Hospital of Guangxi Medical University (Guangxi, China) between January 2007 and September 2020...
June 2023: Experimental and Therapeutic Medicine
#22
Nada Alghamdi, Abdulelah Albaqami, Abdulmajeed Alharbi
Immunocompromised patients are at risk of developing atypical herpes simplex virus (HSV) infection, which can be easily misdiagnosed. We present a case of a 69-year-old female who was receiving methotrexate and tofacitinib for a known case of rheumatoid arthritis. She was admitted to the ICU under neurology care after presenting with status epilepticus secondary to bacterial meningitis. She complained of a group of vesicles on the erythematous base accompanied by a burning sensation, erosions with a hemorrhagic crust that extended onto the vermilion lip, and painful oral mucosa erosion that involve the buccal, palatine, and tongue...
April 2023: Curēus
#23
Feng-Qiao Gao, Jun-Mei Zhang, Cai-Feng Li
Paraneoplastic pemphigus (PNP) is a rare autoimmune skin disease closely related to tumors, characterized by a maculopapular rash with mucosal pain, bronchiole occlusion, and respiratory failure may occur over time, even resulting in death. We report a rare case of a child with autoimmune PNP misdiagnosed as juvenile dermatomyositis (JDM), and summarize the key points of differentiation of clinical manifestations and auxiliary examinations of PNP and JDM. When the diagnosis is not clear because the patient has features not typical of JDM, then skin biopsy and other diagnostic studies should be considered prior to any immunosuppressive therapy, as this could potentially obscure and delay the diagnosis of malignancy...
September 2023: International Journal of Rheumatic Diseases
#24
Phuong Daniels, Yujie Linda Liou, Kelly B Scarberry, Timmie R Sharma, Neil J Korman
No abstract text is available yet for this article.
May 2023: JAAD Case Reports
#25
Mariana Grigore, Mariana Costache, Olga Simionescu
Paraneoplastic pemphigus (PNP) is a rare bullous disease with a polymorphic presentation. Diagnosis can be difficult because it can mimic other bullous diseases, while the underlying neoplasm may be completely asymptomatic. We present the case of a 19-year-old female with a four-year history of exclusively oral bullous lesions, mimicking pemphigus vulgaris, before the diagnosis of a retroperitoneal Castleman disease. While PNP is a severe and sometimes deadly condition, our patient had a mild and long evolution on minimal treatment, with complete resolution after tumor excision...
March 2023: Curēus
#26
Thomas Pagliaro, Benny Yau, Rohan Mortimore, Gregory Butler
No abstract text is available yet for this article.
April 13, 2023: Australasian Journal of Dermatology
#27
Can Chen, Ying Xu, Jingdi Yu, Shenxian Qian, Yaping Xie
Paraneoplastic pemphigus (PNP) is a rare life-threatening disease which always associated with an underlying neoplasm. Tumor-related PNP most commonly precedes the detection of a hematological malignancy, with some cases seen during disease remission following cytotoxic drug therapy or radiotherapy. The lung is the most frequently-involved site in PNP, second only to the eyes, and involvement is seen in 59.2% to 92.8% of PNP cases. Bronchiolitis obliterans (BO) is the end stage of respiratory involvement and is regarded as life-threatening...
2023: Frontiers in Medicine
#28
JOURNAL ARTICLE
Ulrike Raap, Maren M Limberg, Khalaf Kridin, Ralf J Ludwig
Autoimmune bullous skin diseases (AIBDs), such as bullous pemphigoid (BP) and pemphigus, are characterized and caused by autoantibodies targeting structural proteins. In BP, clinical experience and recent systematic evaluation identified pruritus to be common and an important cause of impaired quality of life. Furthermore, chronic pruritus may be the sole clinical symptom of BP. In pemphigus, a retrospective study recently documented a high prevalence of pruritus. The temporal relation between pruritus and BP/pemphigus are, however, unknown...
March 6, 2023: Biomolecules
#29
JOURNAL ARTICLE
Emiliano Antiga, Rikke Bech, Roberto Maglie, Giovanni Genovese, Luca Borradori, Barbara Bockle, Marzia Caproni, Frédéric Caux, Nisha Suyien Chandran, Alberto Corrà, Francesco D'Amore, Maryam Daneshpazhooh, Dipankar De, Dario Didona, Marian Dmochowski, Kossara Drenovska, Jan Ehrchen, Claudio Feliciani, Matthias Goebeler, Richard Groves, Claudia Günther, Sanjeev Handa, Silke C Hofmann, Barbara Horvath, Dimitrios Ioannidis, Hana Jedlickova, Cezary Kowalewski, Khalaf Kridin, Pascal Joly, Yen Loo Lim, Branka Marinovic, Emanual Maverakis, Joost Meijer, Aikaterini Patsatsi, Carlo Pincelli, Catherine Prost, Jane Setterfield, Eli Sprecher, Dusan Skiljevic, Kaisa Tasanen, Soner Uzun, Nina Van Beek, Snejina Vassileva, Artem Vorobyev, Igor Vujic, Gang Wang, Mingyue Wang, Katarzyna Wozniak, Savas Yayli, Giovanna Zambruno, Takashi Hashimoto, Enno Schmidt, José Manuel Mascarò, Angelo Valerio Marzano
BACKGROUND: Paraneoplastic pemphigus (PNP), also called paraneoplastic autoimmune multiorgan syndrome (PAMS), is a rare autoimmune disease with mucocutaneous and multi-organ involvement. PNP/PAMS is typically associated with lymphoproliferative or haematological malignancies, and less frequently with solid malignancies. The mortality rate of PNP/PAMS is elevated owing to the increased risk of severe infections and disease-associated complications, such as bronchiolitis obliterans. OBJECTIVES: These guidelines summarize evidence-based and expert-based recommendations (S2k level) for the clinical characterization, diagnosis and management of PNP/PAMS...
June 2023: Journal of the European Academy of Dermatology and Venereology: JEADV
#30
JOURNAL ARTICLE
Mariangela Irrera, Elena Bozzola, Antonello Cardoni, Rita DeVito, Andrea Diociaiuti, Maya El Hachem, Katia Girardi, Alessandra Marchesi, Alberto Villani
BACKGROUND: In literature, a few reports described an association between paraneoplastic pemphigus (PNP) and Castelman's disease (CD), but no consensus have been proposed for the diagnostic-therapeutical approach. Aim of this study is to present a case report and explore the relationship between PNP and CD in pediatric patients, focusing on clinical manifestations, histopathological findings, treatment and outcome to find elements for an early diagnosis. CASE PRESENTATION: We present the clinical case of a 13 years old girl with a challenging diagnosis of PNP and CD who underwent therapy at first with Rituximab and then with Siltuximab, for the control of symptoms...
March 20, 2023: Italian Journal of Pediatrics
#31
JOURNAL ARTICLE
Mohammad Majid Paracha, Farah Sagheer, Abdul Qayyum Khan
A seven-year retrospective study was held at the Department of Dermatology, Lady Reading Hospital, Peshawar, between 2013 to 2020 to determine the demography and clinical features of pemphigus. Among 148 patients included in this study 88 (58%) were females and 60 (40%) were males with a female to male ratio of 1.46:1. Average age at onset of the disease was 38±12 years (range 14-75 years). On the basis of Autoimmune Bullous Skin Disorder Score (ABSIS), 14 (9.3%) patients had mild disease, 58 (38.7%) had moderate disease, and 76 (50...
March 2023: JPMA. the Journal of the Pakistan Medical Association
#32
JOURNAL ARTICLE
Giulia Gasparini, Emanuele Cozzani, Giovanni Di Zenzo, Adele Salemme, Eva Dematté, Camilla Vassallo, Angelo Valerio Marzano, Giovanni Genovese, Marzia Caproni, Emiliano Antiga, Pietro Quaglino, Aurora Parodi
BACKGROUND: Mucous membrane pemphigoid (MMP) with anti-laminin 332 autoantibodies may be associated with malignancies, however, current serological assays have considerable limitations. At present, no commercial test for anti-laminin 332 antibodies is available, restricting the diagnosis to specialized laboratories worldwide. Biochip immunofluorescence microscopy has shown promising results in selected cohorts of laminin 332-MMP patients. OBJECTIVES: To detect anti-laminin 332 antibodies by biochip immunofluorescence microscopy in a real-life cohort of MMP patients and compare the results with those from traditional immunoblotting...
November 1, 2022: European Journal of Dermatology: EJD
#33
Maria Relvas, Joana Xará, Miguel Lucas, Sónia Coelho, Inês Coutinho, José C Cardoso, Leonor Ramos
No abstract text is available yet for this article.
January 30, 2023: Journal of Paediatrics and Child Health
#34
REVIEW
L Galicier, N Schleinitz
The spectrum of Castleman disease encompasses several different disorders. Nowadays three different forms of the disease are individualized: unicentric Castleman disease, multicentric HHV-8 associated Castleman disease and idiopathic multicentric Castleman disease. In the latter a severe form called TAFRO syndrome (thrombocytopenia, anasarca, myelofibrosis, renal dysfunction, and organomegaly) tend to be individualized. Improvement in the classification and understanding of the physiopathology of CD have allowed improvement in treatment strategies...
December 2022: La Revue de Médecine Interne
#35
JOURNAL ARTICLE
Dong Ni Leng, Kang-Jie Yu, Jing Wang
BACKGROUND: Inflammatory pseudotumor-like follicular dendritic cell sarcoma (IPT-like FDCS) is often associated with Epstein-Barr (EB) virus infection. The tumor is commonly found in the spleen and liver, and it has been reported in the literature that it can be associated with paraneoplastic pemphigus, myasthenia gravis, and other diseases. A case of IPT-like FDCS with clinical features of thrombocytopenia has not been reported. PATIENT CONCERNS: A 59-year-old male patient visited our hospital in September 2020 due to bleeding gums and epistaxis...
December 30, 2022: Medicine (Baltimore)
#36
REVIEW
Hayato Takahashi, Hisato Iriki, Yasuhiko Asahina
Pemphigus is a life-threatening autoimmune bullous disease mediated by anti-desmoglein IgG autoantibodies. Pemphigus is mainly classified into three subtypes: pemphigus vulgaris, pemphigus foliaceus, and paraneoplastic pemphigus. The pathogenicity of autoantibodies has been extensively studied. Anti-human CD20 antibody therapy targeting B cells emerged as a more effective treatment option compared to conventional therapy for patients with an intractable disease. On the other hand, autoreactive T cells are considered to be involved in the pathogenesis based on the test results of human leukocyte antigen association, autoreactive T cell detection, and cytokine profile analysis...
December 20, 2022: Journal of Dermatology
#37
JOURNAL ARTICLE
Erkan Alpsoy
No abstract text is available yet for this article.
January 2023: Journal of the European Academy of Dermatology and Venereology: JEADV
#38
JOURNAL ARTICLE
C Wei, T H He, J L Zhuang, Daobin Zhou
No abstract text is available yet for this article.
December 1, 2022: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
#39
Wasin Saowaluksakul, Chutima Seree-Aphinan, Suthinee Rutnin, Kochawan Boonyawat, Kumutnart Chanprapaph
Pemphigus and lupus erythematosus are both B-cell-mediated autoimmune diseases, dependent on autoreactive CD4+ T lymphocytes to modulate autoimmune B-cell response. Many forms of pemphigus have been reported to occur in association with systemic lupus erythematosus (SLE) as well as other autoimmune diseases. However, it remains unclear whether this association occurs because of a shared immunopathogenesis or the coexistence may be coincidental. We hereby present a case report of discoid lupus erythematosus and paraneoplastic pemphigus associated with marginal zone lymphoma in a 54-year-old Thai man who had persistent oral erosions for 1 year together with generalized polymorphic cutaneous eruptions for 2 months...
2022: Clinical, Cosmetic and Investigational Dermatology
#40
Blake O Adnani, Kathleen O' Brien, Zaw W Myint, Brandon L Adler
A 49-year-old male with no past medical history presented with acute-onset painful mucosal erosions along with flaccid bullae on his trunk, scalp, and intertriginous areas. The patient initially underwent a skin biopsy which demonstrated suprabasilar acantholysis and lichenoid interface dermatitis. This was followed by a computed tomography scan which identified a large abdominal lymph node. Core needle biopsy of this node demonstrated follicular lymphoma. Lastly, indirect immunofluorescence (IIF) in rat bladder was positive (titer 1 : 10,240)...
2022: Case Reports in Hematology
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