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https://www.readbyqxmd.com/read/27899204/stevens-johnson-syndrome-in-childhood
#1
N Blanco, B Gutiérrez, I Valls, D Puertas, C Martín, M Rivera, Á Hernández, A Torrelo
INTRODUCTION: Stevens-Johnson syndrome and toxic epidermal necrolysis are two serious immune diseases within the context of bullous mucocutaneous syndrome. These have varying degrees of involvement of the skin and usually at least two mucous membranes. CLINICAL CASES: Three clinical cases are presented, two of them with significant ophthalmological sequelae, who had received drug treatment as a possible trigger, and another milder clinical case caused by Mycoplasma pneumoniae...
November 26, 2016: Archivos de la Sociedad Española de Oftalmología
https://www.readbyqxmd.com/read/27893467/bullous-pyoderma-gangrenosum-with-subungual-involvement-associated-with-ulcerative-colitis
#2
Ezgi Aktaş Karabay, Asl Aksu Cerman, İlknur Kvanc Altunay, Özben Yalçn
Pyoderma gangrenosum (PG) is a rare inflammatory and ulcerative skin disease of unknown etiology characterized by neutrophilic infiltration of the dermis, mainly affecting the lower extremities. Bullous PG is a rare variant of this disease, usually associated with hematologic disorders. Here, we report a case of pathergy-positive bullous PG with subungual involvement associated with ulcerative colitis.
November 22, 2016: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/27885526/twelve-year-follow-up-of-penetrating-keratoplasty
#3
Takashi Ono, Sosuke Ishiyama, Takeshi Hayashidera, Yosai Mori, Ryohei Nejima, Kazunori Miyata, Shiro Amano
PURPOSE: To evaluate the long-term outcomes of penetrating keratoplasty (PKP) according to the corneal disease diagnosis and the number of PKP procedures performed. METHODS: Five-hundred-and-nine eyes from 403 patients who underwent PKP at Miyata Eye Hospital in Japan from 1998 through 2014, were included in this study. Medical charts were retrospectively examined to ascertain the corneal disease diagnosis and the period of graft survival. Graft survival rates were compared among various corneal disease diagnoses and among the number of PKP procedures performed...
November 24, 2016: Japanese Journal of Ophthalmology
https://www.readbyqxmd.com/read/27878477/paraneoplastic-pemphigus-and-autoimmune-blistering-diseases-associated-with-neoplasm-characteristics-diagnosis-associated-neoplasms-proposed-pathogenesis-treatment
#4
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...
November 22, 2016: American Journal of Clinical Dermatology
https://www.readbyqxmd.com/read/27878308/-systemic-lupus-erythematosus-unusual-cutaneous-manifestations
#5
REVIEW
T Stockinger, L Richter, M Kanzler, M Melichart-Kotik, H Pas, K Derfler, E Schmidt, K Rappersberger
BACKGROUND: Various different mucocutaneous symptoms may affect up to 80 % of systemic lupus erythematosus (SLE) patients. OBJECTIVES: To investigate, various unspecific, but otherwise typical clinical symptoms of skin and mucous membranes that arise in SLE patients other than those defined as SLE criteria such as butterfly rash, chronic cutaneous lupus erythematosus, oral ulcers, and increased photosensitivity. MATERIALS AND METHODS: Extensive search of peer-reviewed scientific articles was performed, medical histories of several SLE patients seen in our department were analyzed, and the rare disease courses in three SLE patients are presented...
December 2016: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
https://www.readbyqxmd.com/read/27876358/a-randomized-double-blind-trial-of-intravenous-immunoglobulin-for-bullous-pemphigoid
#6
Masayuki Amagai, Shigaku Ikeda, Takashi Hashimoto, Masato Mizuashi, Akihiro Fujisawa, Hironobu Ihn, Yasushi Matsuzaki, Mikio Ohtsuka, Hiroshi Fujiwara, Junichi Furuta, Osamu Tago, Jun Yamagami, Akiko Tanikawa, Hisashi Uhara, Akimichi Morita, Gen Nakanishi, Mamori Tani, Yumi Aoyama, Eiichi Makino, Masahiko Muto, Motomu Manabe, Takayuki Konno, Satoru Murata, Seiichi Izaki, Hideaki Watanabe, Yukie Yamaguchi, Setsuko Matsukura, Mariko Seishima, Koji Habe, Yuichi Yoshida, Sakae Kaneko, Hajime Shindo, Kimiko Nakajima, Takuro Kanekura, Kenzo Takahashi, Yasuo Kitajima, Koji Hashimoto
BACKGROUND: Patients with steroid-resistant bullous pemphigoid (BP) require an appropriate treatment option. OBJECTIVE: A multicenter, randomized, placebo-controlled, double-blind trial was conducted to investigate the therapeutic effect of high-dose intravenous immunoglobulin (IVIG; 400mg/kg/day for 5days) in BP patients who showed no symptomatic improvement with prednisolone (≥0.4mg/kg/day) administered. METHODS: We evaluated the efficacy using the disease activity score on day15 (DAS15) as a primary endpoint, and changes in the DAS over time, the anti-BP180 antibody titer, and safety for a period of 57days as secondary endpoints...
November 9, 2016: Journal of Dermatological Science
https://www.readbyqxmd.com/read/27874879/bullous-pemphigoid-associated-with-a-lymphoepithelial-cyst-of-the-pancreas
#7
Preston W Chadwick, Francis R Spitz, Daniel M Kwa, Waine C Johnson, Warren R Heymann
Bullous pemphigoid (BP) is an acquired, autoimmune, subepidermal blistering disorder. A possible paraneoplastic association has been suggested; however, debate remains regarding the precise relationship of these neoplasms with BP. We present a case of recalcitrant BP in a 67-year-old man with a pancreatic neoplasm that was found to be a lymphoepithelial cyst. Immunoperoxidase staining of the cyst demonstrated C3d along the basement membrane of the stratified squamous epithelium, suggesting that the BP may have involved the lymphoepithelial cyst itself...
October 2016: Cutis; Cutaneous Medicine for the Practitioner
https://www.readbyqxmd.com/read/27869429/-topical-corticosteroids-as-a-therapeutic-alternative-in-linear-immunoglobulin-a-bullous-dermatosis-in-childhood-case-report
#8
Francisco José Gil Sáenz, Gabriel Durán Urdániz, Marta Fernández Galar, Juan Gimeno Ballester, Ana Herrero Varasa, Rosa Garcés Bordege
Linear immunoglobulin A dermatosis of childhood is a rare autoimmune disorder. Its etiology remains unknown, although it has been linked to drugs, infections, immunological diseases and lymphoproliferative processes. We report the case of a 6 year old girl who consulted for perioral bullous lesions without other symptoms. Neither treatment with mupirocin nor methylprednisolone therapy achieved remission of cutaneous lesions. Skin biopsy showed a linear immunoglobulin A dermatosis. It was not possible to start treatment with dapsone because of a partial glucose-6-phosphate dehydrogenase deficiency, so topical treatment was maintained with good evolution of lesions...
December 1, 2016: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/27868136/increased-activity-and-apoptosis-of-eosinophils-in-blister-fluids-skin-and-peripheral-blood-of-patients-with-bullous-pemphigoid
#9
Judith Engmann, Urda Rüdrich, Georg Behrens, Eleni Papakonstantinou, Manuela Gehring, Alexander Kapp, Ulrike Raap
Bullous pemphigoid (BP) is an autoimmune blistering skin disease that is more common in elderly individuals. The aim of this study was to determine the functional activity of eosinophils in patients with BP compared with healthy donors. Blood, skin and blister-derived eosinophils were strongly activated in patients with BP, seen by increased surface expression of CD69 compared with controls. CD11b was also increased in BP blood eosinophils, which may explain the striking accumulation of eosinophils in BP (1×10-6 per ml blister fluid)...
November 21, 2016: Acta Dermato-venereologica
https://www.readbyqxmd.com/read/27867744/iga-pemphigus-showing-iga-antibodies-to-desmoglein-1-and-3
#10
Salama Hegazy, Sana Bouchouicha, Aida Khaled, Lilia Laadher, Maryem Kallel Sellami, Faten Zeglaoui
BACKGROUND: IgA pemphigus is a rare autoimmune vesiculo-pustular skin disease. Only approximately 70 cases have been reported to date. We report a case of IgA pemphigus with IgA antibodies to desmoglein 1 (Dsg1) and desmoglein 3 (Dsg3). CASE REPORT: We report the case of an 60-year-old man with intraepidermal neutrophilic IgA pemphigus with IgA antibodies to Dsg1 and Dsg3. Histologic examination revealed subcorneal neutrophilic pustules with few acantholytic cells...
October 2016: Dermatology Practical & Conceptual
https://www.readbyqxmd.com/read/27867571/role-of-oxidative-stress-and-outcome-of-various-surgical-approaches-among-patients-with-bullous-lung-disease-candidate-for-surgical-interference
#11
Ahmed Farouk, Mohammed H Hassan, Mohammed Alaa Nady, Mohammed Farouk Abdel Hafez
BACKGROUND: Bullous lung disease is characterized by formation of blebs, bullae and emphysema. We investigate the role of oxidative stress in the pathogenesis of bullous lung disease and compare between conventional thoracotomy versus video assisted thoracoscopic approach in surgical management of such patients. METHODS: This study was a prospective case control study and it was carried out on 21 patients (16 males and 5 females) with bullous lung disease selected as candidate for surgical interference...
October 2016: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/27845416/psychiatric-and-neurological-disorders-are-associated-with-bullous-pemphigoid-a-nationwide-finnish-care-register-study
#12
Anna-Kaisa Försti, Jari Jokelainen, Hanna Ansakorpi, Allan Seppänen, Kari Majamaa, Markku Timonen, Kaisa Tasanen
Bullous pemphigoid (BP) is an autoimmune blistering skin disease with increasing incidence. BP is associated with neurological disorders, but it has not been established, what subtypes of dementia and stroke are associated with BP, and what is the temporal relation between these diseases. Also, the association between BP and psychiatric disorders is controversial. We conducted a retrospective nationwide study, using the Finnish Care Register for Health Care diagnoses between 1987 and 2013. The study population of 4524 BP patients were compared with 66138 patients with basocellular carcinoma (BCC), neurological and psychiatric comorbid disorders were evaluated for both groups, and associations were estimated by Cox regression and logistic regression analyses...
November 15, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27837194/lenalidomide-for-refractory-cutaneous-manifestations-of-pediatric-systemic-lupus-erythematosus
#13
E Y Wu, L E Schanberg, E C Wershba, C E Rabinovich
OBJECTIVE: Cutaneous manifestations of pediatric systemic lupus erythematosus cause significant morbidity. Lenalidomide, a thalidomide analogue, has shown promise treating cutaneous lupus erythematosus in adults. Our objective was to evaluate lenalidomide's efficacy and safety in treating refractory cutaneous manifestations of pediatric systemic lupus erythematosus. METHODS: We performed a retrospective chart review of 10 adolescents who received lenalidomide for recalcitrant cutaneous lupus erythematosus...
November 10, 2016: Lupus
https://www.readbyqxmd.com/read/27829102/correlation-of-serum-levels-of-ige-autoantibodies-against-bp180-with-bullous-pemphigoid-disease-activity
#14
Nina van Beek, Nadine Lüttmann, Franziska Huebner, Andreas Recke, Ingolf Karl, Franziska Sophie Schulze, Detlef Zillikens, Enno Schmidt
Importance: Bullous pemphigoid (BP) is by far the most frequent autoimmune blistering disease. The presence of IgE autoantibodies against the transmembrane protein BP antigen 2 (BP180, type XVII collagen) has previously been reported in 22% to 100% of BP serum samples, and the pathogenic relevance of anti-BP180 IgE has been suggested in various experimental models and by the successful use of omalizumab in individual patients with BP. Objectives: To determine the rate of anti-BP180-reactive IgE in BP, to evaluate the diagnostic relevance of anti-BP180 IgE in BP, and to correlate anti-BP180 IgE with disease activity and the clinical phenotype of patients with BP...
November 9, 2016: JAMA Dermatology
https://www.readbyqxmd.com/read/27828632/quality-of-life-depression-anxiety-and-loneliness-in-patients-with-bullous-pemphigoid-a-case-control-study
#15
Anargyros Kouris, Eftychia Platsidaki, Christos Christodoulou, Kalliopi Armyra, Panagiota Korkoliakou, Christina Stefanaki, Revekka Tsatovidou, Dimitrios Rigopoulos, George Kontochristopoulos
Background: Bullous pemphigoid (BP) is a chronic, autoimmune blistering skin disease that affects patients' daily life and psychosocial well-being. Objective: The aim of the study was to evaluate the quality of life, anxiety, depression and loneliness in BP patients. Methods: Fifty-seven BP patients and fifty-seven healthy controls were recruited for the study. The quality of life of each patient was assessed using the Dermatology Life Quality Index (DLQI) scale...
September 2016: Anais Brasileiros de Dermatologia
https://www.readbyqxmd.com/read/27822733/-cutaneous-manifestations-in-renal-diseases
#16
M Schmid-Simbeck, A Udvardi, B Volc-Platzer
In addition to general skin changes like pallor or dryness and the frequent, often excruciating nephrogenic pruritus, specific diseases in patients with renal failure may occur. Acquired perforating dermatoses are usually also highly pruritic. Calciphylaxis is a severe disease with poor prognosis. Nonhealing wounds with superinfection and progression to sepsis are characteristic. Bullous lesions can be caused by disturbances in porphyrin metabolism. Nephrogenic systemic fibrosis is a disease which was first described in 2000...
December 2016: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
https://www.readbyqxmd.com/read/27820951/elevated-cytokine-levels-in-the-aqueous-humor-of-eyes-with-bullous-keratopathy-and-low-endothelial-cell-density
#17
Takefumi Yamaguchi, Kazunari Higa, Terumasa Suzuki, Naohiko Nakayama, Yukari Yagi-Yaguchi, Murat Dogru, Yoshiyuki Satake, Jun Shimazaki
Purpose: To evaluate cytokine levels in the aqueous humor (AqH) of eyes with bullous keratopathy (BK) and low endothelial cell density (ECD). Methods: A total of 145 AqH samples (60 BK, 16 low ECD, 35 corneal diseases with normal ECD, and 34 normal controls) were collected from consecutive patients who underwent corneal transplantation or cataract surgery. None of the patients had any clinically apparent inflammation at the time of AqH collection. The AqH levels of cytokines (IL-1α, IL-1β, IL-2, IL-4, IL-6, IL-8, IL-10, IL-12p70, IL-13, IL-17A, IFN-α, IFN-γ, monocyte chemotactic protein [MCP]-1, TNF-α, E-selectin, P-selectin, soluble intercellular adhesion molecule [sICAM]-1, granulocyte-macrophage colony-stimulating factor [GM-CSF], macrophage inflammatory protein [MIP] -1α and MIP-1β) were compared among the groups...
November 1, 2016: Investigative Ophthalmology & Visual Science
https://www.readbyqxmd.com/read/27814422/development-of-bullous-pemphigoid-in-a-patient-with-psoriasis-and-metabolic-syndrome
#18
Anna Lesniewska, Agnieszka Kalińska-Bienias, Cezary Kowalewski, Robert Schwartz, Katarzyna Wozniak
Bullous pemphigoid (BP) is an autoimmune subepidermal blistering disease that most commonly affects adults older than 60 years, whereas psoriasis vulgaris (PV) is a chronic immune-mediated disease that affects both children and adults. Bullous pemphigoid and PV may coexist with each other as well as with various other internal disorders, which may lead to early death. We report the case of a 35-year-old man with a 15-year history of PV and obesity who developed tense blisters with annular arrangement and normal-appearing perilesional skin localized mainly on the trunk, arms, and legs resembling linear IgA bullous dermatosis...
September 2016: Cutis; Cutaneous Medicine for the Practitioner
https://www.readbyqxmd.com/read/27811462/delayed-diagnosis-of-bullous-pyoderma-gangrenosum-with-acute-myelogenous-leukemia
#19
S Sener, G Sarac, N Altunisik, Y Bayindir
Pyoderma gangrenosum (PG) is a rare, but serious neutrophilic dermatosis characterized by recurrent painful cutaneous ulcerations. It is commonly associated with inflammatory bowel disease, rheumatoid arthritis, and hematological malignancies. Because laboratory evaluations and histologic features of PG are nonspecific, diagnosis is based on the clinical features of the ulcer and requires exclusion of other conditions that cause such ulceration. The disease responds to glucocorticoids, immunosuppressives, and anti-inflammatory drugs...
November 2016: Nigerian Journal of Clinical Practice
https://www.readbyqxmd.com/read/27792825/features-of-oral-pharyngeal-and-laryngeal-lesions-in-bullous-pemphigoid
#20
Masafumi Ohki, Shigeru Kikuchi, Atsushi Ohata, Yuka Baba, Junichi Ishikawa, Hirohito Sugimoto
Bullous pemphigoid is an autoimmune bullous disease characterized by skin lesions, with or without oral lesions. The occurrence of pharyngolaryngeal lesions is very rare in affected patients. We conducted a study to investigate the characteristics of oral and pharyngolaryngeal lesions in bullous pemphigoid. Our study population was made up of 6 consecutively presenting outpatients-2 men and 4 women, aged 40 to 83 years (mean: 68.2)-who had been referred to our department over an 11-year period. Presenting symptoms included sore throat in all 6 patients and oral pain in 3...
October 2016: Ear, Nose, & Throat Journal
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