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https://www.readbyqxmd.com/read/28718937/wound-healing-in-epidermolysis-bullosa
#1
L B Tuderman, J E Mellerio
Epidermolysis bullosa (EB) is a group of inherited diseases characterised by recurrent skin blistering due to impaired epidermal or dermo-epidermal adhesion. In some subtypes, damage to internal organs causes serious comorbidities, and an increased risk of early and aggressive squamous cell carcinoma (SCC) characterizes recessive dystrophic EB (RDEB). It is likely that EB will only be cured by mutation-correction interventions, but until that goal becomes a reality we need to pool and extend knowledge about wound management and other strategies to improve quality of life for people with EB...
July 18, 2017: British Journal of Dermatology
https://www.readbyqxmd.com/read/28711083/bedside-diagnostics-in-dermatology-parasitic-and-noninfectious-diseases
#2
REVIEW
Robert G Micheletti, Arturo R Dominguez, Karolyn A Wanat
In addition to aiding the diagnosis of viral, bacterial, and fungal diseases, mineral oil preparation, Tzanck smear, and other techniques can be used to diagnose parasitic infections, neonatal pustular dermatoses, blistering diseases, Stevens-Johnson syndrome, and a plethora of other benign and malignant conditions, including granulomatous diseases and tumors. In many cases, these techniques are specific, reliable, and easy to perform and interpret. In others, a certain amount of training and expertise are required...
August 2017: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/28697212/maxillary-implant-prosthodontic-treatment-using-digital-laboratory-protocol-for-a-patient-with-epidermolysis-bullosa-a-case-history-report
#3
Rubén Agustín-Panadero, Blanca Serra-Pastor, David Peñarrocha-Oltra, Miguel Peñarrocha-Diago
Epidermolysis bullosa belongs to a group of genetic diseases that present with skin disorders and is characterized by generalized blister formation in response to mechanical trauma. This article reports on the management of a recessive dystrophic epidermolytic patient with four remaining periodontally compromised maxillary teeth. Treatment involved placement of four maxillary implants and use of computer-aided design/computer-assisted manufacture techniques to fabricate a fixed full-arch implant-supported prosthesis...
July 2017: International Journal of Prosthodontics
https://www.readbyqxmd.com/read/28691931/gentamicin-induces-functional-type-vii-collagen-in-recessive-dystrophic-epidermolysis-bullosa-patients
#4
David T Woodley, Jon Cogan, Yingping Hou, Chao Lyu, M Peter Marinkovich, Douglas Keene, Mei Chen
BACKGROUND: Recessive dystrophic epidermolysis bullosa (RDEB) is an incurable disease caused by mutations in the gene encoding type VII collagen, the major component of anchoring fibrils (AF). We previously demonstrated that gentamicin produced functional type VII collagen in RDEB cells harboring nonsense mutations. Herein, we determined whether topical or intradermal gentamicin administration induces type VII collagen and AFs in RDEB patients. METHODS: A double-blind, placebo-controlled pilot trial assessed safety and efficacy of topical and intradermal gentamicin in 5 RDEB patients with nonsense mutations...
July 10, 2017: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/28684769/eosinophil-cationic-protein-ecp-a-predictive-marker-of-bullous-pemphigoid-severity-and-outcome
#5
Delphine Giusti, Gregory Gatouillat, Sébastien Le Jan, Julie Plée, Philippe Bernard, Frank Antonicelli, Bach Nga Pham
Bullous Pemphigoid (BP) is an inflammatory rare autoimmune bullous dermatosis, which outcome cannot be predicted through clinical investigations. Eosinophils are the main immune infiltrated cells in BP. However, the release of Major Basic Protein (MBP), Eosinophil Derived Neurotoxin (EDN), and Eosinophil Cationic Protein (ECP) upon eosinophil activation has still not been evaluated with respect to BP development. MBP, EDN and ECP were measured by ELISA in serum (n = 61) and blister fluid (n = 20) of patients with BP at baseline, and in serum after 2 months of treatment (n = 41)...
July 6, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28681645/mean-platelet-volume-in-pemphigus-vulgaris
#6
Khalaf Kridin, Wesal Shihade, Shira Zelber-Sagi
Pemphigus vulgaris (PV) is an autoimmune blistering disease of the skin and mucous membranes. The mean platelet volume (MPV) is an index of platelet activation reported to be influenced by inflammation. We investigated the MPV in patients with PV relative to controls. This case-control study included 160 patients with PV and 640 age- and sex-matched controls. The MPV, platelet count, and other inflammatory parameters were measured for all study participants. A significantly decreased MPV was observed in patients with PV compared with controls, 9...
January 1, 2017: Angiology
https://www.readbyqxmd.com/read/28680463/diagnosis-and-treatment-of-herpes-simplex-1-virus-infection-in-pregnancy
#7
REVIEW
Rachel Lee, Manju Nair
A nulliparous woman presented at 21 weeks' gestation with a 72-h history of a rash on her left arm. Initially isolated to the forearm, it had quickly spread, becoming multiple itchy fluid-filled blisters. Blood tests showed mild neutrophilia and raised CRP. Skin swabs demonstrated the presence of herpes simplex virus type 1 (HSV1) DNA. There was no history of previous HSV1 exposure. There is scant literature on uncomplicated cutaneous HSV1 since the majority is oral/genital. The incidence of transmission varies and is dependent on site of infection and immunological status...
June 2017: Obstetric Medicine
https://www.readbyqxmd.com/read/28676329/multiple-and-repeated-sampling-increases-the-sensitivity-of-direct-immunofluorescence-testing-for-the-diagnosis-of-mucous-membrane-pemphigoid
#8
Iakov Shimanovich, Julia Marie Nitz, Detlef Zillikens
BACKGROUND: Mucous membrane pemphigoid (MMP) is an autoimmune disease characterized by the predominant blistering of mucosal surfaces and the linear deposition of complement, IgG, or IgA along the basement membrane detected by direct immunofluorescence (DIF) test. OBJECTIVE: To assess the impact of multiple and repeated DIF sampling on establishing the diagnosis of MMP. METHODS: We reviewed the results of DIF studies in 136 nonlesional biopsies from 78 patients who were immunologically confirmed to have MMP...
July 1, 2017: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/28676094/cultured-epidermal-stem-cells-in-regenerative-medicine
#9
REVIEW
Catherine J Jackson, Kim Alexander Tønseth, Tor Paaske Utheim
Transplantation of cultured epidermal cell sheets (CES) has long been used to treat patients with burns, chronic wounds, and stable vitiligo. In patients with large area burns this can be a life-saving procedure. The ultimate goal, however, is to restore all normal functions of the skin and prevent scar formation. Increased focus on the incorporation of epidermal stem cells (EpiSCs) within CES transplants may ultimately prove to be key to achieving this. Transplanted EpiSCs contribute to restoring the complete epidermis and provide long-term renewal...
July 4, 2017: Stem Cell Research & Therapy
https://www.readbyqxmd.com/read/28672992/inhibition-of-viral-replication-by-small-interfering-rna-targeting-of-the-foot-and-mouth-disease-virus-receptor-integrin-%C3%AE-6
#10
Na-Na Hu, Wenzhi Zhang, Lina Wang, Yuan-Zhi Wang, Chuang-Fu Chen
In animals, foot-and-mouth disease (FMD) causes symptoms such as fever, limping and the development of blister spots on the skin and mucous membranes. RNA interference (RNAi) may be a novel way of controlling the FMD virus (FMDV), specifically by targeting its cognate receptor protein integrin β6. The present study used RNAi technology to construct and screen plasmids that expressed small interfering RNA molecules (siRNAs) specific for the integrin β6 subunit. Expression of green fluorescence protein from the RNAi plasmids was observed following transfection into porcine embryonic fibroblast (PEF) cells, and RNAi plasmids were screened using reverse transcription-quantitative polymerase chain reaction (RT-qPCR) analysis...
July 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28670357/raising-awareness-among-healthcare-providers-about-epidermolysis-bullosa-and-advancing-toward-a-cure
#11
REVIEW
Aaron Tabor, Joseph V Pergolizzi, Guy Marti, John Harmon, Bernard Cohen, Jo Ann Lequang
Objective: Epidermolysis bullosa (EB) is an orphan disease that affects about half a million people worldwide, but may not be familiar to all clinicians. The authors' goal was to present a short description of this condition and current research in the form of a narrative review. Methods: The authors reviewed the literature on epidermolysis bullosa in order to describe the condition and current genetic research. Results: There are at least 31 subtypes of EB, including junctional EB, dystrophic EB, and Kindler syndrome...
May 2017: Journal of Clinical and Aesthetic Dermatology
https://www.readbyqxmd.com/read/28670245/reviewing-putative-industrial-triggering-in-pemphigus-cluster-of-pemphigus-in-the-area-near-the-wastewater-treatment-plant
#12
REVIEW
Paweł Pietkiewicz, Justyna Gornowicz-Porowska, Paweł Bartkiewicz, Monika Bowszyc-Dmochowska, Marian Dmochowski
A range of pemphigus is relatively rare potentially fatal group of autoimmune blistering dermatoses. Usually, there is no apparent triggering, while in some predisposed patients there are alleged environmental/industrial inducing factors. In a short time period (4 years), we diagnosed 3 novel cases of pemphigus (1 pemphigus vulgaris, 1 pemphigus foliaceus and 1 shift from pemphigus foliaceus into pemphigus vulgaris) at a clinical and laboratory level (ELISA, immunofluorescence studies). We discuss a possible common inducing mechanism as these patients inhabit one estate of the Poznan suburbia (Kozieglowy, population < 12,000), Greater Poland district, Poland, and review literature data on alleged pemphigus triggers...
June 2017: Postȩpy Dermatologii i Alergologii
https://www.readbyqxmd.com/read/28670088/linear-iga-bullous-dermatosis-mimicking-oral-lichen-planus
#13
Sheevam Shah, Brooke Mohr, Palak Parekh
Linear IgA bullous dermatosis (LABD) is an autoimmune disease characterized by blisters on the skin and mucous membranes. Polycyclic, grouped bullae on cutaneous skin are the most characteristic clinical feature of LABD. Lesions are often indistinguishable from bullous pemphigoid or may resemble dermatitis herpetiformis. Oral lesions may be seen in 5% to 70% of patients with LABD, and in some cases, the oral mucosa may be the only area of involvement. Herein, we report a patient with LABD involving only the oral mucosa clinically...
July 2017: Proceedings of the Baylor University Medical Center
https://www.readbyqxmd.com/read/28660618/high-frequency-ultrasound-in-blistering-skin-diseases-a-useful-method-for-differentiating-blister-locations
#14
Xiujun Cheng, Jianke Li, Guizhi Zhou, Yongxia Liu, Xianmei Lu, Na Wang, Hong Liu, Furen Zhang
Bullous pemphigoid and pemphigus vulgaris, which belong to the group of subepidermal and intraepidermal bullae, respectively, are two potentially devastating blistering skin diseases. We used high-frequency ultrasound (US) in 3 cases of these diseases as prototypes to study the value of high-frequency US in discriminating blister locations. Our findings showed that high-frequency US has a strong correlation with histomorphometric findings because of its high resolution, and we hope that it will be helpful for differentiating blister locations...
June 29, 2017: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
https://www.readbyqxmd.com/read/28659052/magnetic-resonance-guided-focused-ultrasound-for-treatment-of-bone-tumors
#15
Vivek Ajit Singh, Syed Usman Shah, Nor Faissal Yasin, Basri Johan Jeet Abdullah
AIMS: Magnetic resonance guided focused ultrasound (MRgFUS) is a new modality in the management of primary and secondary bone tumors. We aimed to investigate the safety, efficacy, and feasibility of using MRgFUS for the treatment of (1) benign bone tumors with the intent of complete tumor ablation, (2) primary malignant bone tumors with the intent to assess its effectiveness in causing tumor necrosis, and (3) metastatic bone disease with the intent of pain relief. METHOD: Twenty-four patients with benign bone tumors, primary malignant bone tumors, and metastatic bone disease were treated with one session of MRgFUS...
May 2017: Journal of Orthopaedic Surgery
https://www.readbyqxmd.com/read/28653439/intraepidermal-neutrophilic-dermatosis-type-of-iga-pemphigus-with-circulating-linear-iga-disease-antibodies-associated-with-ulcerative-colitis
#16
Eleni Papakonstantinou, Alexander Kapp, Marcel F Jonkman, Ulrike Raap
A 42-year-old woman with ulcerative colitis previously well controlled on mesalazine presented with blistering, crusts and severe itching on her upper body and legs together with painful erosions on her conjunctivae and oral mucous membranes in addition to active bowel symptoms for two weeks. Clinical examination revealed multiple lesions consisting of vesiculopustules with circinate distribution and central crusts in sunflower-like configuration on her flanks and legs, a typical characteristic of intraepidermal neutrophilic dermatosis (IEN)-type of IgA pemphigus (Figure 1A and 1C) [1]...
June 27, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28647348/pivotal-role-of-lesional-and-perilesional-t-b-lymphocytes-in-pemphigus-pathogenesis
#17
Huijie Yuan, Shengru Zhou, Zhicui Liu, Weiting Cong, Xiaochun Fei, Weihong Zeng, Haiqin Zhu, Renchao Xu, Ying Wang, Jie Zheng, Meng Pan
Pemphigus is a skin and mucosal membrane-targeting autoimmune bullous disease. Previous studies have demonstrated that circulating anti-desmoglein1/3 antibodies are pathogenic and mediate blister formation. However, the role of infiltrated immune cells in the lesional skin has not been fully investigated. In this study we showed that there existed a large number of B, T lymphocytes and plasma cells in the skin lesions by immunohistochemistry and immunofluorescence staining. In addition, a significantly increased number of Dsg1 and Dsg3-specific B cells could be identified by flow cytometric analysis or ELISPOT assay...
June 21, 2017: Journal of Investigative Dermatology
https://www.readbyqxmd.com/read/28633733/rituximab-in-the-management-of-juvenile-pemphigus-foliaceus
#18
Tiffany Y Loh, Taraneh Paravar
BackgroundPemphigus foliaceus (PF) is a blistering disorder most commonly presenting in middle age. As PF is restricted to the superficial epidermis, it is considered more benign than other pemphigus diseases. However, progression to severe disease is not uncommon. Although rituximab's efficacy has been well-documented in adults with refractory PF, little data is available on its role in adolescents.PurposeWe describe a patient with juvenile PF treated with rituximab and review the literature for similar cases...
June 15, 2017: Dermatology Online Journal
https://www.readbyqxmd.com/read/28632473/a-rare-occurrence-of-epidermolysis-bullosa-acquisita-in-a-patient-with-retroviral-disease
#19
Zambare Uddhao, Chhonkar Aditi, Nayak Chitra, Tambe Swagata
Epidermolysis bullosa acquisita is a chronic subepidermal blistering disease associated with autoimmunity to type-VII collagen within anchoring fibrils located at the dermo-epidermal junction. This entity is rarely reported from India. It can have a variety of presentations. Its occurrence with retroviral disease has rarely been reported. Here, we present a case of epidermolysis bullosa acquisita in a patient with HIV infection.
January 1, 2017: International Journal of STD & AIDS
https://www.readbyqxmd.com/read/28630891/frequency-of-neurological-disorders-in-bullous-pemphigoid-patients-a-cross-sectional-study
#20
Sheida Khosravani, Farhad Handjani, Reyhaneh Alimohammadi, Nasrin Saki
BACKGROUND: Bullous pemphigoid (BP) is an autoimmune subepidermal blistering skin disorder which occurs mostly in the elderly. Several studies have reported an association between BP and neurological disorders (ND). OBJECTIVE: The purpose of this study was to evaluate the association between BP and neurological disorders in Iranian patients. METHODS: In this cross-sectional study, 87 patients with BP were enrolled. They were compared to 184 controls...
2017: International Scholarly Research Notices
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