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https://www.readbyqxmd.com/read/30022934/bullous-pemphigoid-as-an-adverse-reaction-to-pembrolizumab-two-case-reports
#1
Kenneth Thomsen, Jon Diernaes, Trine Heide Øllegaard, Eva Spaun, Christian Vestergaard
Checkpoint inhibitors are novel and promising treatment options for different types of cancer. Programmed cell death 1 (PD-1) inhibitors, such as pembrolizumab, have been shown to significantly raise the survival rates of disseminated malignant melanoma (MM). Autoimmune adverse reactions are very common in checkpoint inhibitors. We present 2 cases of bullous pemphigoid, as adverse reactions to pembrolizumab-treated MM.
May 2018: Case Reports in Dermatology
https://www.readbyqxmd.com/read/30021739/laryngoceles-with-airway-compromise-complicating-mucous-membrane-pemphigoid
#2
Carolyn Wijaya, Lucy Morgan, Daniel Novakovic, Sean Riminton
Mucous membrane pemphigoid (MMP) encompasses a group of autoantibody-mediated, subepithelial blistering diseases, which primarily affect mucosal surfaces including oral, ocular, skin, genital, nasopharyngeal and oesophageal sites. We present a first description of laryngoceles as a manifestation of mucous membrane pemphigoid resulting in dynamic airway closure. Mucosal injury at other sites had previously resulted in pathergy and localised cicatrisation. We discuss successful combined medical and transcutaneous surgical intervention designed to avoid tracheostomy and minimise iatrogenic laryngeal cicatrisation...
July 18, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/30018132/a-case-of-nivolumab-induced-bullous-pemphigoid-review-of-dermatologic-toxicity-associated-with-programmed-cell-death-protein-1-programmed-death-ligand-1-inhibitors-and-recommendations-for-diagnosis-and-management
#3
Adriana T Lopez, Larisa Geskin
Immunotherapy has emerged as a highly effective treatment for numerous cancers. Use of checkpoint inhibitors against various molecules including programmed cell death protein-1 (PD-1), programmed death ligand-1 (PD-L1), and cytotoxic T-lymphocyte-associated protein-4 have become widespread in clinical practice. Compared with conventional chemotherapy, immunotherapy is associated with a unique set of immune reactions known collectively as immune-related adverse events (irAEs). Of known irAEs, cutaneous toxicity is among the most frequently observed in patients treated with immunotherapy...
July 17, 2018: Oncologist
https://www.readbyqxmd.com/read/30007013/bullous-pemphigoid-with-hyperkeratosis-and-palmoplantar-keratoderma-three-cases
#4
Luca Fania, Dario Didona, Valeria Pacifico, Feliciana Mariotti, Naomi De Luca, Damiano Abeni, Cinzia Mazzanti, Giovanni Di Zenzo, Biagio Didona
The clinical features of bullous pemphigoid are extremely polymorphous. Several atypical forms of bullous pemphigoid have been described, and the diagnosis critically relies on immunopathological findings. We describe three bullous pemphigoid patients characterized by palmoplantar keratoderma, diffused hyperkeratotic cutaneous lesions and extremely high levels of immunoglobulin E serum. The diagnosis of bullous pemphigoid should be taken into account in patients presenting diffused hyperkeratotic cutaneous lesions and palmoplantar keratoderma, even in the absence of blisters...
July 14, 2018: Journal of Dermatology
https://www.readbyqxmd.com/read/30004958/infectious-keratitis-after-ocular-surface-stem-cell-transplantation
#5
Albert Y Cheung, Enrica Sarnicola, Medi Eslani, Khaliq H Kurji, Brad M Genereux, Amit Govil, Edward J Holland
PURPOSE: To describe the rate, clinical/microbiological characteristics, and outcomes of infectious keratitis in eyes with limbal stem cell deficiency after ocular surface stem cell transplantation (OSST). METHODS: In this retrospective chart review of 278 eyes that underwent OSST between January 2006 and December 2016, eyes treated for previous infectious keratitis (bacterial, fungal, or viral) were included. Demographics, risk factors, course, microbiological characteristics, and outcomes were assessed...
July 10, 2018: Cornea
https://www.readbyqxmd.com/read/29989877/bullous-pemphigoid-associated-with-ischemic-cerebrovascular-accident-and-dementia-exclusive-blistering-lesions-on-the-upper-hemiparetic-limb
#6
Sebastian Vernal, Tamiris Julio, Fernanda Cruz, Aline Turatti, Norito Ishii, Takashi Hashimoto, Ana Maria Roselino
Bullous pemphigoid (BP) has been associated with neurological disorders (NDs), which has led to the hypothesis that molecular mimicry exists between hemidesmosomal proteins and neuronal peptides. A 79-year-old hemiparetic woman presented with tense bullae affecting exclusively her right paretic upper limb for three months. Histopathology, taken from the perilesional area, revealed an inflammatory infiltrate with predominant eosinophils. IIF evidenced linear IgG deposition in the epidermal side of the cleavage...
June 2018: Acta Dermatovenerologica Croatica: ADC
https://www.readbyqxmd.com/read/29989869/the-value-of-type-iv-collagen-immunohistochemical-staining-in-the-differential-diagnosis-of-autoimmune-subepidermal-bullous-diseases
#7
Ho Yeol Lee, Seung Pil Ham, Yoo Won Choi, Hai-Jin Park
Autoimmune subepidermal bullous diseases (AISBDs) exhibit various clinical presentations, histological appearances, prognoses, and responses to treatment. Many diagnostic techniques, such as direct immunofluorescence (IF), indirect salt-split skin IF, and enzyme-linked immunosorbent assays, are used in the differential diagnoses of AISBDs. However, these techniques require fresh frozen tissue, expensive laboratory equipment, and sophisticated laboratory techniques. The purpose of this study was to evaluate the value of type IV collagen immunohistochemical (IHC) staining for the differential diagnosis of AISBDs...
June 2018: Acta Dermatovenerologica Croatica: ADC
https://www.readbyqxmd.com/read/29988643/pemphigoid-gestationis
#8
Ioannis K Papapanagiotou, Sotiria Tsagouri, Chrysa G Liakou, Alexandros Besharat, Nikistratos Vogiatzis, Konstantinos Ntzeros, Evangelos Petrakis, Pelopidas Koutroumanis, Nikolaos Thomakos, Dimitrios Loutradis
Pemphigoid gestationis is a rare autoimmune skin disorder emerging exclusively during pregnancy. Topical and oral glucocorticoids as well as oral antihistamines are the standard medications administered during pregnancy, aiming to relieve pruritus and to suppress extensive blister formation. Obstetricians should be able to recognize and treat this rare clinical condition accordingly.
July 2018: Clinical Case Reports
https://www.readbyqxmd.com/read/29982604/dystonin-a3-upregulation-is-responsible-for-maintenance-of-tubulin-acetylation-in-a-less-severe-dystonia-musculorum-mouse-model-for-hereditary-sensory-and-autonomic-neuropathy-type-vi
#9
Anisha Lynch-Godrei, Yves De Repentigny, Sabrina Gagnon, My Tran Trung, Rashmi Kothary
Hereditary sensory and autonomic neuropathy type VI (HSAN-VI) is a recessive human disease that arises from mutations in the dystonin gene (DST; also known as Bullous pemphigoid antigen 1 gene [BPAG1]). A milder form of HSAN-VI was recently described, resulting from loss of a single dystonin isoform (DST-A2). Similarly, mutations in the mouse dystonin gene (Dst) result in severe sensory neuropathy, dystonia musculorum (Dstdt). Two Dstdt alleles, Dstdt-Tg4 and Dstdt-27J, differ in the severity of disease. The less severe Dstdt-Tg4 mice have disrupted expression of Dst-A1 and -A2 isoforms, while the more severe Dstdt-27J allele affects Dst-A1, -A2 and -A3 isoforms...
July 4, 2018: Human Molecular Genetics
https://www.readbyqxmd.com/read/29980216/molecular-diagnosis-of-anti-laminin-332-epiligrin-mucous-membrane-pemphigoid
#10
Roxana Chiorean, Sorina Danescu, Oana Virtic, Mayson B Mustafa, Adrian Baican, Annette Lischka, Takashi Hashimoto, Yoshinobu Kariya, Manuel Koch, Cassian Sitaru
BACKGROUND: Mucous membrane pemphigoid is a group of chronic subepithelial autoimmune blistering diseases that mainly affect mucous membranes. Laminin 332-specific autoantibodies are present in approximately 1/3 of the patients, being associated with an increased risk of malignancy. Because of the severe complications, an early recognition of the disease allowing a timely therapy is essential. The gold standard methods for detection of laminin 332-specific autoantibodies, including the immunoprecipitation and immunoblotting are non-quantitative, laborious and restricted to a few specialized laboratories worldwide...
July 6, 2018: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/29977929/autoimmune-diseases-and-their-manifestations-on-oral-cavity-diagnosis-and-clinical-management
#11
REVIEW
Matteo Saccucci, Gabriele Di Carlo, Maurizio Bossù, Francesca Giovarruscio, Alessandro Salucci, Antonella Polimeni
Oral signs are frequently the first manifestation of autoimmune diseases. For this reason, dentists play an important role in the detection of emerging autoimmune pathologies. Indeed, an early diagnosis can play a decisive role in improving the quality of treatment strategies as well as quality of life. This can be obtained thanks to specific knowledge of oral manifestations of autoimmune diseases. This review is aimed at describing oral presentations, diagnosis, and treatment strategies for systemic lupus erythematosus, Sjögren syndrome, pemphigus vulgaris, mucous membrane pemphigoid, and Behcet disease...
2018: Journal of Immunology Research
https://www.readbyqxmd.com/read/29972879/diagnosis-of-anti-laminin-%C3%AE-1-pemphigoid-by-immunoblot-analysis
#12
Farzan Solimani, Robert Pollmann, Norito Ishii, Rüdiger Eming, Takashi Hashimoto, Thomas Schmidt, Michael Hertl
BACKGROUND: Anti-laminin-γ1 (lam-γ1) pemphigoid, a recently described immunobullous disorder sharing immune serological features of bullous pemphigoid and epidermolysis bullosa acquisita (EBA), is characterized by the detection of serum IgG autoantibodies against the lam-γ1 chain, a 200 kDa heterotrimeric component of the dermal-epidermal junction (DEJ). OBJECTIVE: The aim of the study was to develop an easy to perform and reliable assay for the serological detection of anti-lam-γ1 IgG autoantibodies...
July 4, 2018: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/29963683/mortality-in-patients-with-bullous-pemphigoid-a-retrospective-cohort-study-systematic-review-and-meta-analysis
#13
Khalaf Kridin, Reuven Bergman
There is little consensus regarding mortality data in bullous pemphigoid (BP). The aim of this study was to evaluate mortality among a relatively large cohort of Israeli patients with BP and to perform a meta-analysis synthesizing existing data on 1-year mortality rates of patients with BP. This retrospective cohort study of 287 patients diagnosed with BP between 2000 and 2015 compared the mortality of cases with age- and sex-matched control subjects in the general population. The results showed 1-, 5- and 10-year mortality rates of 26...
March 27, 2018: Acta Dermato-venereologica
https://www.readbyqxmd.com/read/29956465/vegetating-erosive-cutaneous-lesions-and-pyogenic-granuloma-in-the-course-of-mucous-membrane-pemphigoid-a-case-report-and-review-of-literature
#14
Beata Jakubowska, Cezary Kowalewski, Norito Ishii, Takashi Hashimoto, Katarzyna Wozniak
Mucous membrane pemphigoid (MMP) is a group of chronic autoimmune sub-epithelial blistering disorders, which mostly affect the oral mucosa and the conjunctiva. MMP is very diverse in terms of both the clinical and immunological features (IgG and IgA autoantibodies may react with different antigens). MMP can be induced by infections and medication, including ophthalmologic medication, which may lead to the development of eye lesions. In contrast, a vegetating variant of MMP is extremely rare. Here, we report an MMP case that demonstrated unusual clinical features, that is, pyogenic granulomas on the conjunctivae and extensive vegetating erosions on the skin of intertriginous regions...
June 29, 2018: International Wound Journal
https://www.readbyqxmd.com/read/29952301/a-screening-programme-for-dermatologists-as-a-guide-to-request-psychological-consultation-in-routine-clinical-practice
#15
Annarita Panebianco, Francesca Sampogna, Maria Luisa Iemboli, Luciano Sobrino, Elisabetta Andreoli, Valeria Antinone, Cinzia Mazzanti, Damiano Abeni
BACKGROUND: The psychosocial impact of skin conditions is usually evaluated in research settings. OBJECTIVES: To define predictors that can be used by dermatologists to refer patients for psychological consultation or psychotherapy using a simple screening instrument. MATERIALS & METHODS: A questionnaire to evaluate possible anxiety and depression (12-item General Health Questionnaire [GHQ-12]) was routinely used over a period of two months in an Italian dermatological reference centre...
June 28, 2018: European Journal of Dermatology: EJD
https://www.readbyqxmd.com/read/29952290/a-case-of-acquired-haemophilia-a-in-a-patient-with-bullous-pemphigoid-and-review-of-the-japanese-literature
#16
Chika Chijiwa, Masahiro Kamata, Atsuko Fukuyasu, Yuki Shono, Shintaro Takeoka, Mihoko Tateishi, Saki Fukaya, Kotaro Hayashi, Takamitsu Tanaka, Takeko Ishikawa, Takamitsu Ohnishi, Koji Saito, Norito Ishii, Takashi Hashimoto, Yayoi Tada
No abstract text is available yet for this article.
June 28, 2018: European Journal of Dermatology: EJD
https://www.readbyqxmd.com/read/29948572/thyroid-diseases-and-skin-autoimmunity
#17
REVIEW
Enke Baldini, Teresa Odorisio, Chiara Tuccilli, Severino Persechino, Salvatore Sorrenti, Antonio Catania, Daniele Pironi, Giovanni Carbotta, Laura Giacomelli, Stefano Arcieri, Massimo Vergine, Massimo Monti, Salvatore Ulisse
The skin is the largest organ of the body, at the boundary with the outside environment. Primarily, it provides a physical and chemical barrier against external insults, but it can act also as immune organ because it contains a whole host of immune-competent cells of both the innate and the adaptive immune systems, which cooperate in eliminating invading pathogens following tissue injury. On the other hand, improper skin immune responses lead to autoimmune skin diseases (AISD), such as pemphigus, bullous pemphigoid, vitiligo, and alopecia...
June 15, 2018: Reviews in Endocrine & Metabolic Disorders
https://www.readbyqxmd.com/read/29948283/haplotypes-of-abcb1-1236c-t-rs1128503-2677g-t-a-rs2032582-and-3435c-t-rs1045642-in-patients-with-bullous-pemphigoid
#18
Mariola Rychlik-Sych, Małgorzata Barańska, Michał Dudarewicz, Jadwiga Skrętkowicz, Agnieszka Żebrowska, Anna Woźniacka, Jacek Owczarek, Daria Orszulak-Michalak, Elżbieta Waszczykowska
Bullous pemphigoid (BP) constitutes the most prevalent disease in the group of bullous dermatoses with the autoimmune background. Some authors suggest that certain cytokines (IL-2, IFN-γ) may be transported by P-glycoprotein (P-gp), the product of the ABCB1 gene. ABCB1 polymorphism might affect not only the effectiveness of treatment with drugs that are P-gp substrates but also contribute to the development of diseases, including BP. In the present work, we resolved to conduct a haplotype analysis of ABCB1 in patients with BP and to answer the question of whether any of the haplotypes are able to affect the incidence of this entity...
June 9, 2018: Archives of Dermatological Research
https://www.readbyqxmd.com/read/29946501/a-case-of-bullous-pemphigoid-a-prevalent-and-potentially-fatal-condition
#19
Jorge Parellada, Yanetsy Olivera Arencibia, Harold Watson, Nicole Parellada, Lara E Saikaly, Sami K Saikaly
Bullous pemphigoid is the most common of the blistering disorders. It is most commonly found in the elderly and is diagnosed based on clinical, histologic, and immunologic criteria. It presents clinically with diffuse eczematous, pruritic, urticaria-like lesions, with the later appearance of tense bullae or blistering lesions typically filled with clear fluid. Histologically, a sub-epidermal blister is seen and immunofluorescence shows immunoglobulin G antibodies directed against the structural components of the keratinocytic hemidesmosomal proteins BP180 and BP230...
April 25, 2018: Curēus
https://www.readbyqxmd.com/read/29946113/granzyme-b-is-elevated-in-autoimmune-blistering-diseases-and-cleaves-key-anchoring-proteins-of-the-dermal-epidermal-junction
#20
Valerio Russo, Theo Klein, Darielle J Lim, Nestor Solis, Yoan Machado, Sho Hiroyasu, Layla Nabai, Yue Shen, Matthew R Zeglinski, Hongyan Zhao, Cameron P Oram, Peter A Lennox, Nancy Van Laeken, Nick J Carr, Richard I Crawford, Claus-Werner Franzke, Christopher M Overall, David J Granville
In healthy skin, epidermis and dermis are anchored together at the dermal-epidermal junction (DEJ), a specialized basement membrane pivotal for skin integrity and function. However, increased inflammation in the DEJ is associated with the disruption and separation of this junction and sub-epidermal blistering. Granzyme B (GzmB) is a serine protease secreted by immune cells. Dysregulated inflammation may lead to increased GzmB accumulation and proteolysis in the extracellular milieu. Although elevated GzmB is observed at the level of the DEJ in inflammatory and blistering skin conditions, the present study is the first to explore GzmB in the context of DEJ degradation in autoimmune sub-epidermal blistering...
June 26, 2018: Scientific Reports
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