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Blistering disease

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https://www.readbyqxmd.com/read/28426486/neutrophilic-figurate-erythema
#1
Yu-Hung Wu, Pa-Fan Hsiao
Neutrophilic figurate erythema (NFE) has been rarely reported. This study aimed to identify the clinical and pathological features of NFE. We retrospectively reviewed the information from diagnostic cases from 2000 to 2013. The diagnosis of NFE includes clinically annular rash, histopathologically predominant neutrophilic perivascular and interstitial infiltrate in the dermis without evidence of vasculitis, and exclusion of other known specific entities. Fifteen cases of NFE were identified, including 11 women and 4 men...
May 2017: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/28418613/complement-independent-blistering-mechanisms-in-bullous-pemphigoid
#2
Hiroaki Iwata, Hideyuki Ujiie
Bullous pemphigoid (BP) is an autoimmune subepidermal blistering disease that clinically demonstrates tense blisters with widespread erythema, histologically demonstrates subepidermal blistering and immunologically demonstrates the presence of circulating autoantibodies against hemidesmosomal molecules. Complement activation has long been regarded as necessary for the generation of the BP. However, certain evidence has recently come to support non-complemental blistering mechanisms. The story of BP blistering mechanisms is a complicated one...
April 18, 2017: Experimental Dermatology
https://www.readbyqxmd.com/read/28418589/a-distinct-cutaneous-microbiota-pofile-in-autoimmune-bullous-disease-patients
#3
Mor Miodovnik, Axel Künstner, Ewan A Langan, Detlef Zillikens, Regine Gläser, Eli Sprecher, John F Baines, Enno Schmidt, Saleh M Ibrahim
Bullous Pemphigoid (BP) is the most common autoimmune blistering disease in Europe. As both the incidence of the disease and the relative proportion of the elderly population continue to rise, it represents a significant medical burden. Whereas some progress has been achieved in defining genetic risk factors for autoimmune blistering diseases, no environmental agent has been conclusively identified. Emerging evidence suggests that host immunity may influence the skin microbiota while the latter modulates cutaneous immunity...
April 18, 2017: Experimental Dermatology
https://www.readbyqxmd.com/read/28412634/chitosan-induced-immunity-in-camellia-sinensis-l-o-kuntze-against-blister-blight-disease-is-mediated-by-nitric-oxide
#4
Swarnendu Chandra, Nilanjan Chakraborty, Koustubh Panda, Krishnendu Acharya
Blister blight disease, caused by an obligate biotrophic fungal pathogen, Exobasidium vexans Massee is posing a serious threat for tea cultivation in Asia. As the use of chemical pesticides on tea leaves substantially increases the toxic risks of tea consumption, serious attempts are being made to control such pathogens by boosting the intrinsic natural defense responses against invading pathogens in tea plants. In this study, the nature and durability of resistance offered by chitosan and the possible mechanism of chitosan-induced defense induction in Camellia sinensis (L...
April 7, 2017: Plant Physiology and Biochemistry: PPB
https://www.readbyqxmd.com/read/28407871/acral-hemorrhagic-darier-disease
#5
M Á Flores-Terry, M García-Arpa, M Llamas-Velasco, C Mendoza-Chaparro, C Ramos-Rodríguez
Darier disease is an autosomal-dominant inherited condition caused by mutation of a gene, which produces a protein involved in calcium channel regulation. The disease has a variety of manifestations and lacks consistent genotype-phenotype correlations. Acral hemorrhagic Darier disease causes macules, papules, vesicles and/or hemorrhagic blisters on the extremities. Other classic signs of the disease may be present in the same patient or relatives. Histopathology reveals dyskeratosis and suprabasal acantholysis with hemorrhagic lacunae...
April 10, 2017: Actas Dermo-sifiliográficas
https://www.readbyqxmd.com/read/28406394/a-randomised-controlled-trial-to-compare-the-safety-effectiveness-and-cost-effectiveness-of-doxycycline-200%C3%A2-mg-day-with-that-of-oral-prednisolone-0-5%C3%A2-mg-kg-day-for-initial-treatment-of-bullous-pemphigoid-the-bullous-pemphigoid-steroids-and-tetracyclines-blister
#6
Joanne R Chalmers, Fenella Wojnarowska, Gudula Kirtschig, James Mason, Margaret Childs, Diane Whitham, Karen Harman, Anna Chapman, Shernaz Walton, Enno Schmidt, Thomas R Godec, Andrew J Nunn, Hywel C Williams
BACKGROUND: Bullous pemphigoid (BP) is an autoimmune blistering skin disorder with increased morbidity and mortality in the elderly. OBJECTIVES: To evaluate the effectiveness, safety and cost-effectiveness of a strategy of initiating BP treatment with oral doxycycline or oral prednisolone. We hypothesised that starting treatment with doxycycline gives acceptable short-term blister control while conferring long-term safety advantages over starting treatment with oral prednisolone...
March 2017: Health Technology Assessment: HTA
https://www.readbyqxmd.com/read/28393203/increased-mir-424-5p-expression-in-peripheral-blood-mononuclear-cells-from-patients-with-pemphigus
#7
Menglei Wang, Liuping Liang, Li Li, Kai Han, Qian Li, Yusheng Peng, Xuebiao Peng, Kang Zeng
Pemphigus is an autoimmune disease that causes blisters and erosions in the skin and mucous membranes. The development of pemphigus is associated with the imbalance of T‑cell and humoral responses. MicroRNAs (miRNAs) can regulate many cell functions. However, whether miRNA expression is altered in peripheral blood mononuclear cells (PBMCs) during the pathogenesis of pemphigus has not been clarified. The aim of the present study was to examine the miRNA expression profiles of PBMCs from patients with pemphigus...
April 3, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28392652/paraneoplastic-pemphigus-associated-with-a-malignant-thymoma-a-case-of-persistent-and-refractory-oral-ulcerations-following-thymectomy
#8
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/28390814/meeting-report-of-the-pathogenesis-of-pemphigus-and-pemphigoid-meeting-in-munich-september-2016
#9
Enno Schmidt, Volker Spindler, Rüdiger Eming, Masayuki Amagai, Frank Antonicelli, John F Baines, Meriem Belheouane, Philippe Bernard, Luca Borradori, Marzia Caproni, Giovanni Di Zenzo, Sergei Grando, Karen Harman, Marcel F Jonkman, Hiroshi Koga, Ralf J Ludwig, Andrew P Kowalczyk, Eliane J Müller, Wataru Nishie, Hendri Pas, Aimee S Payne, Christian D Sadik, Allan Seppänen, Jane Setterfield, Hiroshi Shimizu, Animesh A Sinha, Eli Sprecher, Michael Sticherling, Hideyuki Ujiie, Detlef Zillikens, Michael Hertl, Jens Waschke
Autoimmune blistering diseases are a heterogeneous group of about a dozen complex disorders that are characterized by intraepidermal (pemphigus) and subepidermal blistering (pemphigoid diseases and dermatitis herpetiformis). The Pathogenesis of Pemphigus and Pemphigoid Meeting, organized by the Departments of Dermatology in Lübeck and Marburg and the Institute of Anatomy and Cell Biology, Munich, was held in September 2016 in Munich. The meeting brought together basic scientists and clinicians from all continents dedicating their work to autoimmune blistering diseases...
April 5, 2017: Journal of Investigative Dermatology
https://www.readbyqxmd.com/read/28381691/autoimmune-bullous-disease-and-hashimoto-s-disease-complicated-by-acquired-hemophilia-a
#10
Nobuko Nishiura, Daisuke Ujimoto, Jiro Fujita, Tetsuo Maeda, Yukinobu Nakagawa, Hirokazu Kashiwagi, Kenji Oritani, Yoshiaki Tomiyama, Yuzuru Kanakura
A 67-year-old man was admitted with a 1-month history of spontaneous hematoma in his right back and severe anemia. He had suffered from rashes with blisters involving both legs for 10 years, which had shown worsening and extended to his entire body concurrently with the hematoma. APTT was markedly prolonged to 119 seconds, and Factor VIII:C and FVIII inhibitor levels were less than 1% and 153.1 BU/ml, respectively, confirming the diagnosis of acquired hemophilia A (AHA). Skin biopsy revealed his rashes to be caused by autoimmune bullous disease (ABD), and laboratory and physical findings showed that he also had autoimmune hypothyroidism (Hashimoto's disease)...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28381287/munchausen-by-proxy-syndrome-mimicking-systemic-autoinflammatory-disease-case-report-and-review-of-the-literature
#11
Helmut Wittkowski, Claas Hinze, Sigrid Häfner-Harms, Vinzenz Oji, Katja Masjosthusmann, Martina Monninger, Ulrike Grenzebach, Dirk Foell
BACKGROUND: Systemic autoinflammatory diseases (SAIDs) represent a growing number of monogenic, polygenic or multifactorial disorders that are often difficult to diagnose. CASE PRESENTATION: Here we report a patient who was initially erroneously diagnosed and treated for SAID. Symptoms consisted of recurrent fever, erythematous and/or blistering skin lesions, angioedema, susceptibility to bleeding, external ear infections and reversible anisocoria in the absence of laboratory evidence of systemic inflammation...
April 5, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28378419/successful-treatment-of-pemphigus-foliaceus-in-a-berrichon-du-cher-ram-with-methylprednisolone-acetate
#12
Bianca Lambacher, Julia Schoiswohl, Rene Brunthaler, Thomas Wittek, Reinhild Krametter-Frötscher
BACKGROUND: Pemphigus foliaceus is a severe, autoimmune blistering skin disease, which is described in humans and some animal species. In small ruminants pemphigus foliaceus has rarely been described and, to the best of the authors' knowledge, little information is available about successful treatment in sheep. AIM: This case report describes a Berrichon du Cher ram with the presumed diagnosis of pemphigus foliaceus. METHODS: The ram was treated with methylprednisolone acetate 40 mg at a dosage of 2...
April 4, 2017: Veterinary Dermatology
https://www.readbyqxmd.com/read/28373119/photodynamic-therapy-effective-for-the-treatment-of-actinic-keratosis-and-basal-cell-carcinoma-in-bullous-pemphigoid-patients
#13
Theresa N Canavan, Salma Faghri de la Feld, Conway Huang, Naveed Sami
Treating skin cancers and extensive actinic keratosis in patients with bullous pemphigoid (BP) can be challenging. Treatment options pose unique risks in these patients as surgical wounds can have delayed wound healing and photodynamic therapy (PDT) may exacerbate their blistering disease. We report the successful use of PDT to treat actinic keratosis and skin cancers in two patients with BP, both of whom had excellent response to PDT and tolerated treatment without any bullous disease flares. Carefully selected patients with skin cancers and stable, well controlled BP can be safely considered for treatment using PDT...
March 31, 2017: Photodiagnosis and Photodynamic Therapy
https://www.readbyqxmd.com/read/28368874/efficacy-of-triaging-direct-immunofluorescence-in-intraepidermal-bullous-dermatoses
#14
Brian J Tjarks, Steven D Billings, Jennifer S Ko
BACKGROUND: Direct immunofluorescence (DIF) is considered pivotal in diagnosing autoimmune blistering diseases. Our goal was to examine the necessity of DIF in intraepidermal bullous cases, of which pemphigus vulgaris (PV) is the prototype. METHODS: Sixty-six cases from 2010 to 2014 submitted for DIF with an intraepidermal blistering disease listed in the differential diagnosis were reviewed by 2 board-certified dermatopathologists to see if they would order DIF based on routine histologic findings...
March 24, 2017: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/28366912/low-dose-rituximab-as-an-adjuvant-therapy-in-pemphigus
#15
Jaya Gupta, Ranjan C Raval, Arti N Shah, Rekha B Solanki, Dhara D Patel, Kaksha B Shah, Ami D Badheka, Keyur B Shah, Neetish K Aggarwal, Vaaruni Ravishankar
BACKGROUND: Pemphigus is a chronic autoimmune blistering disease where systemic steroids and immunosuppressants are the mainstay of therapy, but long-term treatment with these agents is associated with many side effects. Rituximab, a chimeric monoclonal anti-CD20 antibody, in low doses has shown efficacy as an adjuvant to reduce the dose of steroids. AIM: To study the clinical efficacy and safety of low-dose rituximab as an adjuvant therapy in pemphigus. METHODS: Fifty patients with extensive pemphigus were selected, who either had recalcitrant pemphigus, were steroid dependent, had relapsed after pulse therapy, had anti-desmoglein levels >20, had contraindications to conventional treatment or wanted to avoid conventional treatment and its side effects...
March 29, 2017: Indian Journal of Dermatology, Venereology and Leprology
https://www.readbyqxmd.com/read/28357176/novel-and-emerging-therapies-in-the-treatment-of-recessive-dystrophic-epidermolysis-bullosa
#16
REVIEW
Ellie Rashidghamat, John A McGrath
Epidermolysis bullosa (EB) is a clinically and genetically heterogeneous group of inherited blistering diseases that affects ∼ 500,000 people worldwide. Clinically, individuals with EB have fragile skin and are susceptible to blistering following minimal trauma, with mucous membrane and other organ involvement in some subtypes. Within the spectrum of EB, ∼ 5% of affected individuals have the clinically more severe recessive dystrophic (RDEB) variant with a prevalence of 8 per one million of the population...
February 2017: Intractable & Rare Diseases Research
https://www.readbyqxmd.com/read/28352068/systemic-lupus-erythematosus-and-bullous-pemphigoid-with-dramatic-response-to-dapsone
#17
Maria Cristina Maggio, Giovanni Corsello, Eugenia Prinzi, Rolando Cimaz
BACKGROUND Bullous pemphigoid is an autoimmune blistering disease, with relapses, isolated or associated with other autoimmune diseases such as systemic lupus erythematosus (SLE). Joint manifestations rapidly respond to small or moderate doses of corticosteroids, whereas skin manifestations usually respond to antimalarial drugs. CASE REPORT We describe the clinical case of an 11-year-old girl with SLE. She showed bullous skin lesions with arthralgia, mild proteinuria, resolved after steroid treatment. At the tapering of her prednisone dose, the patient had new skin lesions requiring an increased dose of prednisone...
March 29, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28348966/cassia-fistula-a-remedy-from-traditional-persian-medicine-for-treatment-of-cutaneous-lesions-of-pemphigus-vulgaris
#18
REVIEW
Fatemeh Atarzadeh, Mohammad Kamalinejad, Ladan Dastgheib, Gholamreza Amin, Amir Mohammad Jaladat, Majid Nimrouzi
OBJECTIVE: Pemphigus is a rare autoimmune disease that may be fatal without proper medical intervention. It is a blistering disease that involves both the skin and mucus membranes, in which the most important causes of death comprise superimposed opportunistic infections and complications of long-term high-dose corticosteroid therapy or prolonged consumption of immune suppressant drugs. Skin lesions are the most important sources of infection, and any local treatment decreasing the healing time of lesions and reducing the total dosage of drugs is favorable...
March 2017: Avicenna Journal of Phytomedicine
https://www.readbyqxmd.com/read/28342533/eosinophilic-dermatosis-of-hematologic-malignancy
#19
S Lucas-Truyols, B Rodrigo-Nicolás, C Lloret-Ruiz, E Quecedo-Estébanez
Dermatosis characterized by tissue eosinophilia arising in the context of hematologic disease is known as eosinophilic dermatosis of hematologic malignancy. The most commonly associated malignancy is chronic lymphocytic leukemia. Eosinophilic dermatosis of hematologic malignancy is a rare condition with a wide variety of clinical presentations, ranging from papules, erythematous nodules, or blisters that simulate arthropod bites, to the formation of true plaques of differing sizes. Histology reveals the presence of abundant eosinophils...
March 22, 2017: Actas Dermo-sifiliográficas
https://www.readbyqxmd.com/read/28338219/human-orf-complicated-by-epidermolysis-bullosa-acquisita
#20
E Zuelgaray, C Salle de Chou, J Gottlieb, M Battistella, M D Vignon-Pennamen, M Bagot, F Guibal, J D Bouaziz
Orf is a DNA parapoxvirus transmitted to humans by contact with infected goats and sheep. Many complications have been reported after Orf infection including erythema multiforme. A few cases of auto-immune bullous dermatosis complicating Orf disease have been reported to date, usually characterized by tense blisters eruptions with or without mucosal involvement, linear deposition of C3, IgG and/or IgA along the basement membrane and negativity of indirect immunofluorescence analysis and ELISA assays (performed in 4 of 11 reported cases) against target antigens of bullous pemphigoid, mucous membrane pemphigoid or epidermolysis bullosa acquisita, except one case of mucosal pemphigoid with antilaminin-332 antibodies...
March 24, 2017: British Journal of Dermatology
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