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https://www.readbyqxmd.com/read/29664511/bullous-henoch-sch%C3%A3-nlein-purpura-case-report
#1
Trinidad Hasbún, Ximena Chaparro, Viera Kaplan, Felipe Cavagnaro, Alex Castro
Henoch-Schönlein purpura (HSP) or IgA Vasculitis is the most common childhood vasculitis. The classic tetrad of signs and symptoms include palpable purpura, arthralgia, abdominal pain and renal disease. The occurrence of hemorrhagic bullae in children with HSP is rarely encountered. Objec tive: To report an unusual cutaneous manifestation of HSP in children. CASE REPORT: A 14-year-old girl complained about a 2-week painful bullous rash in both lower extremities and multiple arthral gias...
February 2018: Revista Chilena de Pediatría
https://www.readbyqxmd.com/read/29663327/peripheral-eosinophilia-in-bullous-pemphigoid-prevalence-and-influence-on-the-clinical-manifestation
#2
K Kridin
BACKGROUND: Peripheral eosinophilia has been reported in 50-60% of bullous pemphigoid (BP) patients and correlated positively with disease severity. The association of peripheral eosinophilia with the different morphological characteristics of BP and the presence of tissue eosinophilia has not been established. METHODS: The study was designed as a case-control study. The diagnosis of BP was grounded on the well-established immunopathological criteria. Five age-, sex-, and ethnicity-matched control subjects were randomly selected for each BP patient...
April 16, 2018: British Journal of Dermatology
https://www.readbyqxmd.com/read/29662486/mucosal-involvement-in-bullous-pemphigoid-is-mostly-associated-with-disease-severity-and-to-absence-of-anti-bp230-autoantibody
#3
Ariane Clapé, Céline Muller, Grégory Gatouillat, Sébastien Le Jan, Coralie Barbe, Bach-Nga Pham, Frank Antonicelli, Philippe Bernard
Bullous pemphigoid (BP) is the most common autoimmune bullous disease and typically affects the elderly. Binding of specific autoantibodies to BP180/230 hemidesmosomal components induces an inflammatory response leading to skin blister formation. Unusual manifestations of BP include additional mucous membrane involvement, without pathophysiological knowledge associated to the formation of these lesions. We here performed a prospective study on series of consecutive BP patients with ( n  = 77) and without ( n  = 18) mucosal involvements at baseline to further investigate why some BP patients display mucosal lesion and other not...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29657064/-selective-left-mainstem-bronchial-intubation-in-the-neonatal-intensive-care-unit
#4
Anthony M H Ho, Michael P Flavin, Melinda L Fleming, Glenio Bitencourt Mizubuti
BACKGROUND: Selective neonatal left mainstem bronchial intubation to treat right lung disease is typically achieved with elaborate maneuvers, instrumentation and devices. This is often attributed to bronchial geometry which favors right mainstem entry of an endotracheal tube deliberately advanced beyond the carina. CASE SUMMARY: A neonate with severe bullous emphysema affecting the right lung required urgent non-ventilation of that lung. We achieved left mainstem bronchial intubation by turning the endotracheal tube 180° such that the Murphy's eye faced the left instead of the right, and simulated a left-handed intubation by slightly orientating the endotracheal tube such that its concavity faced the left instead of the right as in a conventional right-handed intubation...
April 12, 2018: Revista Brasileira de Anestesiologia
https://www.readbyqxmd.com/read/29619987/comorbidities-of-bullous-pemphigoid-in-a-finnish-cohort
#5
Anna Pankakoski, Harri Sintonen, Annamari Ranki, Nicolas Kluger
The incidence of bullous pemphigoid (BP) is increasing in Finland. To investigate the clinical presentation, comorbidities, and medications in a cohort of Finnish patients with confirmed BP managed in a university hospital setting. An observational retrospective study of all consecutive patients diagnosed with BP in 2012-2013 at the Department of dermatology, HUCH. The prevalence of the most common comorbidities was compared to that in a sample population aged over 30 years. Seventy patients were included (mean age: 77 years at diagnosis)...
April 5, 2018: European Journal of Dermatology: EJD
https://www.readbyqxmd.com/read/29619029/anti-type-vii-collagen-antibodies-are-identified-in-a-subpopulation-of-bullous-pemphigoid-patients-with-relapse
#6
Delphine Giusti, Grégory Gatouillat, Sébastien Le Jan, Julie Plée, Philippe Bernard, Frank Antonicelli, Bach-Nga Pham
Bullous pemphigoid (BP) is an autoimmune bullous skin disease characterized by anti-BP180 and anti-BP230 autoantibodies (AAbs). Mucous membrane involvement is an uncommon clinical feature of BP which may evoke epidermolysis bullosa acquisita, another skin autoimmune disease characterized by anti-type VII collagen AAbs. We therefore evaluated the presence of anti-type VII collagen AAbs in the serum of BP patients with and without mucosal lesions at time of diagnosis and under therapy. Anti-BP180, anti-BP230, and anti-type VII collagen AAbs were measured by ELISA in the serum of unselected patients fulfilling clinical and histo/immunopathological BP criteria at baseline ( n  = 71) and at time of relapse ( n  = 24)...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29611499/case-report-paederus-dermatitis-in-the-returning-traveler
#7
Jacob W Pierce, Barry Rittman, Jillian E Raybould
Rash in the returned traveler can provide a diagnostic dilemma for clinicians, but a detailed history including epidemiologic exposures can allow prompt diagnosis of etiologies uncommon in the United States. One such disease is Paederus dermatitis, an irritant contact dermatitis related to exposure to the rove beetle characterized by bullous lesions with surrounding erythema. Although cases and outbreaks have commonly been reported throughout the world, they are rarely reported in travelers returning to the United States...
April 2, 2018: American Journal of Tropical Medicine and Hygiene
https://www.readbyqxmd.com/read/29607480/prospective-study-in-bullous-pemphigoid-association-of-high-serum-anti-bp180-igg-levels-with-increased-mortality-and-reduced-karnofsky-score
#8
M M Holtsche, S Goletz, N van Beek, D Zillikens, S Benoit, K Harman, S Walton, J English, M Sticherling, A Chapman, N J Levell, R Groves, H C Williams, I R König, E Schmidt
BACKGROUND: Bullous pemphigoid (BP) is a subepidermal blistering disease characterized by autoantibodies against the two hemidesmosomal proteins BP180 (type XVII collagen) and BP230. The multicenter prospective BLISTER (Bullous Pemphigoid Steroids and Tetracyclines) trial randomized 253 BP patients to compare the benefits and harms between initial treatment with doxycycline or prednisolone. OBJECTIVES: To analyze distinct autoantibody profiles for the prediction of the disease course in a well characterized cohort of BP sera...
April 1, 2018: British Journal of Dermatology
https://www.readbyqxmd.com/read/29600832/bullous-pemphigoid-italian-guidelines-adapted-from-edf-eadv-guidelines
#9
Emanuele Cozzani, Angelo V Marzano, Marzia Caproni, Claudio Feliciani, Piergiacomo Calzavara-Pinton
Bullous pemphigoid is the most common autoimmune subepidermal blistering disease of the skin and mucous membranes. This disease typically affects the elderly and presents with itch and localised or generalised bullous lesions. In up to 20% of affected patients blister may be completely absent, and only excoriations, prurigo-like lesions, eczematous lesions, urticated lesions, and/or infiltrated plaques are observed. The disease is significantly associated with neurological disorders. The morbidity of bullous pemphigoid and its impact on the quality of life are significant...
March 30, 2018: Giornale Italiano di Dermatologia e Venereologia: Organo Ufficiale, Società Italiana di Dermatologia e Sifilografia
https://www.readbyqxmd.com/read/29599777/tissue-destruction-in-bullous-pemphigoid-can-be-complement-independent-and-may-be-mitigated-by-c5ar2
#10
Christian M Karsten, Tina Beckmann, Maike M Holtsche, Jenny Tillmann, Sabrina Tofern, Franziska S Schulze, Eva Nina Heppe, Ralf J Ludwig, Detlef Zillikens, Inke R König, Jörg Köhl, Enno Schmidt
Bullous pemphigoid (BP), the most frequent autoimmune bullous disorder, is a paradigmatic autoantibody-mediated disease associated with autoantibodies against BP180 (type XVII collagen, Col17). Several animal models have been developed that reflect important clinical and immunological features of human BP. Complement activation has been described as a prerequisite for blister formation, however, the recent finding that skin lesions can be induced by anti-Col17 F(ab')2 fragments indicates complement-independent mechanisms to contribute to blister formation in BP...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29595653/type-i-gaucher-disease-with-bullous-pemphigoid-and-parkinson-disease-a-case-report
#11
Damien Le Peillet, Virginie Prendki, Véronique Trombert, Emmanuel Laffitte, Frédéric Assal, Jean Luc Reny, Christine Serratrice
RATIONALE: Gaucher disease (GD) is a rare genetic lysosomal storage disorder inherited in an autosomal recessive pattern. GD is due to the deficiency of a lysosomal enzyme, acid beta-glucosidase (or glucocerebrosidase). Type 1 Gaucher disease (GD1) is characterized by thrombocytopenia, anemia, an enlarged spleen, and liver as well as bone complications (Erlenmeyer flask deformity, osteoporosis, lytic lesions, pathological and vertebral fractures, bone infarcts, and avascular necrosis leading to degenerative arthropathy)...
March 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29588155/identification-of-immunodominant-th2-cell-epitopes-in-chinese-bullous-pemphigoid-patients
#12
Jieyu Zhang, Hui Fang, Shengxian Shen, Erle Dang, Qiuju Li, Pei Qiao, Hongjiang Qiao, Gang Wang
Bullous pemphigoid (BP) is a subepidermal autoimmune blistering disease caused by autoantibodies (autoAbs) targeting the juxtamembranous extracellular noncollagenous 16A (NC16A) domain of human collagen XVII (also known as BP180). Because T helper cells are essential for antibody responses to antigens, we adopted an assay to map the immunodominant Th2-cell epitopes in NC16A. We synthesized 22 overlapping peptides spanning the entire sequence of BP180-NC16A and investigated the reactivity of Th2 cells from BP patients to these peptides using the ELISPOT assay...
March 24, 2018: Journal of Investigative Dermatology
https://www.readbyqxmd.com/read/29566927/blistering-diseases-in-the-mature-patient
#13
Ines Lakoš Jukić, Sandra Jerković Gulin, Branka Marinović
Autoimmune blistering diseases (AIBD) are a group of chronic diseases affecting the skin and mucous membranes, with different presentation, clinical course, histologic and immunopathologic findings, and different therapeutic approach. Blisters develop as a result of autoantibodies directed against distinct adhesion structures within desmosomes or within the basement membrane zone. The most common AIBD that develops in the elderly is bullous pemphigoid (previously also named "pemphigoid senilis"), but mature patients can also present with other AIBD as mucous membrane pemphigoid, epidermolysis bullosa acquisita, paraneoplastic pemphigus, pemphigus vulgaris, pemphigus foliaceus, linear IgA dermatosis, and dermatitis herpetiformis...
March 2018: Clinics in Dermatology
https://www.readbyqxmd.com/read/29562822/vascular-mapping-for-the-decision-of-hemodialysis-access-method-case-report
#14
Takashi Uchino, Hiromasa Nakai, Hiroaki Ichihashi, Tsukasa Sasaki, Nakanobu Azuma
Preoperative ultrasound vascular mapping was performed to determine the surgical method for two cases where standard arteriovenous fistula was not possible. In case 1, the previous arteriovenous fistula of the left arm was occluded, and arteriovenous graft of the right arm was thrombosed after frequent percutaneous transluminal angioplasty. Contrast medium was contraindicated because of allergy. Using preoperative ultrasound vascular mapping, we were able to establish the appropriate surgical method. In case 2, hemodialysis was started by catheter insertion, and then access creation was planned...
March 1, 2018: Journal of Vascular Access
https://www.readbyqxmd.com/read/29545809/the-autoimmune-skin-disease-bullous-pemphigoid-the-role-of-mast-cells-in-autoantibody-induced-tissue-injury
#15
REVIEW
Hui Fang, Yang Zhang, Ning Li, Gang Wang, Zhi Liu
Bullous pemphigoid (BP) is an autoimmune and inflammatory skin disease associated with subepidermal blistering and autoantibodies directed against the hemidesmosomal components BP180 and BP230. Animal models of BP were developed by passively transferring anti-BP180 IgG into mice, which recapitulates the key features of human BP. By using these in vivo model systems, key cellular and molecular events leading to the BP disease phenotype are identified, including binding of pathogenic IgG to its target, complement activation of the classical pathway, mast cell degranulation, and infiltration and activation of neutrophils...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29541076/the-role-of-mast-cells-in-autoimmune-bullous-dermatoses
#16
REVIEW
Xinhua Yu, Anika Kasprick, Karin Hartmann, Frank Petersen
Skin mast cells (MCs), a resident immune cell type with broad regulatory capacity, play an important role in sensing danger signals as well as in the control of the local immune response. It is conceivable to expect that skin MCs regulate autoimmune response and are thus involved in autoimmune diseases in the skin, e.g., autoimmune bullous dermatoses (AIBD). Therefore, exploring the role of MCs in AIBD will improve our understanding of the disease pathogenesis and the search for novel therapeutic targets. Previously, in clinical studies with AIBD, particularly bullous pemphigoid, patients' samples have demonstrated that MCs are likely involved in the development of the diseases...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29540887/neonatal-purpura-fulminans-a-rare-genetic-disorder-due-to-protein-c-deficiency-a-case-report
#17
Sayyeda Ghazala Irfan Kazi, Emaduddin Siddiqui, Irfan Habib, Saadia Tabassum, Badar Afzal, Irum Qamar Khan
Neonatal Purpura Fulminans is a rare and fatal disorder associated with perivascular haemorrhage and disseminated intravascular coagulation. Early clinical recognition, timely investigation and treatment is utmost important. A 6 days old baby boy was brought to emergency with blackish ulcers all over the body. Initially these were over the feet and scalp but later appeared on the abdomen. On examination, child was vitally stable, mildly icteric and had multiple erythematous large bullous blackish lesions on scalp, lower abdomen, perineum, back and soles...
March 2018: JPMA. the Journal of the Pakistan Medical Association
https://www.readbyqxmd.com/read/29531608/bullous-tinea-incognito-in-a-bulgarian-child-first-description-in-the-medical-literature
#18
Georgi Tchernev, Ivan Terziev
For the first time in the world medical literature, we describe a rare form of cutaneous dermatophytosis - a bullous form of Tinea incognito, classified by clinical picture, histopathological findings and an isolated infectious agent from the microbiological culture. After a thorough review of Medline/PubMed's relevant literature, we could not find similar cases of patients with Tinea incognito who are clinically presented with bullous lesions at the same time. Local application of corticosteroids in infants with unknown lesions may lead to progression of the underlying disease and may cause some serious problems in differential diagnosis aspect, while the clinical expression remains completely masked...
February 15, 2018: Open Access Macedonian Journal of Medical Sciences
https://www.readbyqxmd.com/read/29528002/descemet-membrane-endothelial-keratoplasty-outcomes-in-the-first-year-of-experience
#19
Dilek D Altınörs, Leyla Asena
OBJECTIVES: We aimed to report the clinical outcomes of Descemet membrane endothelial keratoplasty in our first year of experience. MATERIALS AND METHODS: Patients who underwent Descemet membrane endothelial keratoplasty at the Baskent University Faculty of Medicine, Department of Ophthalmology, between 2015 and 2016 were included in the study. Patient demographics, cause of endothelial dysfunction, best-corrected visual acuity, central corneal thickness, graft survival, follow-up duration, and intraoperative and postoperative complications were recorded...
March 2018: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/29527848/corticosteroid-therapy-in-combination-with-antibiotics-for-erysipelas
#20
Michal Solomon, Aviv Barzilai, Hila Elphasy, Henri Trau, Sharon Baum
BACKGROUND: Erysipelas, an acute infection of the dermal and subcutaneous tissue, is normally treated with antibiotics. Previous data indicated that treatment with prednisone in combination with antibiotics results in significant acceleration of the healing phase. OBJECTIVES: To investigate the effectiveness of corticosteroids combined with antibiotics for the treatment of erysipelas. METHODS: A retrospective study was conducted on hospitalized patients diagnosed with erysipelas between 2004 and 2011 at the Department of Dermatology at Sheba Medical Center, Israel...
March 2018: Israel Medical Association Journal: IMAJ
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