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https://www.readbyqxmd.com/read/28719980/distinguishing-epidermolysis-bullosa-acquisita-from-bullous-pemphigoid-without-direct-immunofluorescence
#1
Kerry M Gardner, Richard I Crawford
BACKGROUND: It has been postulated that periodic acid-Schiff staining of basement membrane can predict direct immunofluorescence patterns seen in epidermolysis bullosa acquisita and bullous pemphigoid. It has also been suggested that the type of inflammatory infiltrate or presence of fraying of basal keratinocytes may differentiate these two conditions. OBJECTIVE: In this study, we aimed to confirm these observations. METHODS: We reviewed 13 cases of direct immunofluorescence-confirmed epidermolysis bullosa acquisita and 19 cases of direct immunofluorescence-confirmed bullous pemphigoid, all with a subepidermal blister in the routinely processed specimen...
July 1, 2017: Journal of Cutaneous Medicine and Surgery
https://www.readbyqxmd.com/read/28718937/wound-healing-in-epidermolysis-bullosa
#2
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/28705276/evaluation-of-autoimmune-bullous-diseases-in-elderly-patients-in-iran-a-10-year-retrospective-study
#3
Maryam Ghiasi, Maryam Daneshpazhooh, Muhammadkhuja Ismonov, Cheyda Chams-Davatchi
Autoimmune bullous diseases (ABDs) are uncommon but significant skin disorders with relatively high morbidity and mortality. Some surveys have been carried out to describe the spectrum of ABDs in a region, but this is the first that has focused on ABDs in elderly patients. This study was conducted to determine the clinicoepidemiologic features of ABDs in elderly patients. Medical records of all ABD patients with disease onset after the age of 60 years who presented to the Autoimmune Bullous Diseases Research Center, Tehran, Iran between April 2003 and March 2013 were reviewed...
2017: Skinmed
https://www.readbyqxmd.com/read/28697212/maxillary-implant-prosthodontic-treatment-using-digital-laboratory-protocol-for-a-patient-with-epidermolysis-bullosa-a-case-history-report
#4
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/28693719/type-xvii-collagen-coordinates-proliferation-in-the-interfollicular-epidermis
#5
Mika Watanabe, Ken Natsuga, Wataru Nishie, Yasuaki Kobayashi, Giacomo Donati, Shotaro Suzuki, Yu Fujimura, Tadasuke Tsukiyama, Hideyuki Ujiie, Satoru Shinkuma, Hideki Nakamura, Masamoto Murakami, Michitaka Ozaki, Masaharu Nagayama, Fiona M Watt, Hiroshi Shimizu
Type XVII collagen (COL17) is a transmembrane protein located at the epidermal basement membrane zone. COL17 deficiency results in premature hair aging phenotypes and in junctional epidermolysis bullosa. Here, we show that COL17 plays a central role in regulating interfollicular epidermis (IFE) proliferation. Loss of COL17 leads to transient IFE hypertrophy in neonatal mice owing to aberrant Wnt signaling. The replenishment of COL17 in the neonatal epidermis of COL17-null mice reverses the proliferative IFE phenotype and the altered Wnt signaling...
July 11, 2017: ELife
https://www.readbyqxmd.com/read/28691931/gentamicin-induces-functional-type-vii-collagen-in-recessive-dystrophic-epidermolysis-bullosa-patients
#6
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/28690212/focal-adhesions-in-the-skin-lessons-learned-from-skin-fragility-disorders
#7
Cristina Has, Yinghong He
Focal adhesions are large multiprotein cell-matrix adhesion complexes, which regulate multiple cellular functions, such as adhesion and migration. Their biological significance in skin is underscored by two genetic disorders, the Kindler syndrome and the interstitial lung disease, nephrotic syndrome and epidermolysis bullosa, in which mutations affect focal adhesion proteins, kindlin-1 and the integrin α3 subunit, respectively. Here we provide an overview of what we learned from the study of the molecular mechanisms of these diseases...
June 1, 2017: European Journal of Dermatology: EJD
https://www.readbyqxmd.com/read/28686345/multiple-milia-as-an-isolated-skin-manifestation-of-dominant-dystrophic-epidermolysis-bullosa-evidence-of-phenotypic-variability
#8
(no author information available yet)
No abstract text is available yet for this article.
July 2017: Pediatric 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/28670357/raising-awareness-among-healthcare-providers-about-epidermolysis-bullosa-and-advancing-toward-a-cure
#10
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/28669251/an-algorithmic-approach-for-the-management-of-hand-deformities-in-dystrophic-epidermolysis-bullosa
#11
Serhan Tuncer, Billur Sezgin, Basar Kaya, Suhan Ayhan, Osman Latifoglu
Treatment of hand deformities in epidermolysis bullosa patients represents a challenging field in hand surgery practice, thus a systematic approach by a team is mandatory for a successful result. A simple and practical algorithm for the surgical treatment of hand deformities in EB was employed by the authors where the deformities of each digit in EB patients was categorized according to pseudosyndactyly and interphalangeal joint contracture severity for guidance during the surgical treatment. The current study retrospectively reviewed the medical records and photographic data of 13 EB patients followed in our department, for whom a systematic approach to the management and treatment was used...
July 2, 2017: Journal of Plastic Surgery and Hand Surgery
https://www.readbyqxmd.com/read/28659151/pain-and-quality-of-life-evaluation-in-patients-with-localized-epidermolysis-bullosa-simplex
#12
Jennifer Brun, Christine Chiaverini, Caroline Devos, Stéphanie Leclerc-Mercier, Juliette Mazereeuw, Emmanuelle Bourrat, Annabel Maruani, Stéphanie Mallet, Claire Abasq, Alice Phan, Pierre Vabres, Ludovic Martin, Christine Bodemer, Sylvie Lagrange, Jean-Philippe Lacour
BACKGROUND: A localized form of epidermolysis bullosa simplex (EBS-l) is considered one of the mildest forms of epidermolysis bullosa (EB), with blisters limited to the palms and soles. However, these lesions can be very painful. The aim of the study was to characterize pain in patients with EBS-l and evaluate its impact on quality of life (QoL). Patients were contacted via the Research Group of the French Society of Pediatric Dermatology and the association of EB patients (DEBRA France)...
June 28, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28647894/keratin-gene-mutations-influence-the-keratinocyte-response-to-dna-damage-and-cytokine-induced-apoptosis
#13
Tina Zupancic, Gregor Sersa, Hans Törmä, Ellen Birgitte Lane, Harald Herrmann, Radovan Komel, Mirjana Liovic
The keratin filament cytoskeleton is vital to the normal function of epithelial cells. It provides structural support and regulates different aspects of cell metabolism. Mutations in keratins 5 and 14 cause a skin fragility disorder, epidermolysis bullosa simplex (EBS). Patients with severe EBS have an increased cumulative risk for basal cell carcinoma. In this study, we tested how keratin 5 and 14 mutant EBS patient-derived keratinocytes behave in the face of two different types of stressors that are able to induce cell death: ionizing radiation and cytokines TNF-α and TRAIL...
June 24, 2017: Archives of Dermatological Research
https://www.readbyqxmd.com/read/28640738/evaluation-of-the-effect-and-mechanism-of-action-of-local-phenytoin-in-treatment-of-vitiligo
#14
Asmaa Gaber Abdou, Mohammed Abdelwahed Gaber, Nada Farag Elnaidany, Ayat Elnagar
There are many theories explaining vitiligo such as genetic, autoimmune, neural, free radicals, biochemical, intrinsic defect, melanocytorrhagy and convergent theories. Phenytoin is a widely used anticonvulsant, which is used in cutaneous medicine for treatment of ulcers and epidermolysis bullosa. The aim of this study is to assess the effectiveness of topical phenytoin gel in treatment of vitiligo patients and explaining the underlying mechanism using immunohistochemistry for evaluation of HMB45, CD4 and CD8...
June 22, 2017: Journal of Immunoassay & Immunochemistry
https://www.readbyqxmd.com/read/28632473/a-rare-occurrence-of-epidermolysis-bullosa-acquisita-in-a-patient-with-retroviral-disease
#15
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/28627382/bpag1-a-distinctive-role-in-skin-and-neurological-diseases
#16
REVIEW
Arshad Ali, Lifang Hu, Fan Zhao, Wuxia Qiu, Pai Wang, Xiaoli Ma, Yan Zhang, Lei Chen, Airong Qian
Spectraplakins are multifunctional cytoskeletal linker proteins that act as important communicators, connecting cytoskeletal components with each other and to cellular junctions. Bullous pemphigoid antigen 1 (BPAG1)/dystonin is a member of spectraplakin family and expressed in various tissues. Alternative splicing of BPAG1 gene produces various isoforms with unique structure and domains. BPAG1 plays crucial roles in numerous biological processes, such as cytoskeleton organization, cell polarization, cell adhesion, and cell migration as well as signaling transduction...
June 13, 2017: Seminars in Cell & Developmental Biology
https://www.readbyqxmd.com/read/28615054/end-stage-kidney-disease-in-patient-with-epidermolysis-bullosa-what-are-the-treatment-options-case-report
#17
Michał Małecki, Maciej Domański, Kazimierz Ciechanowski
BACKGROUND: Epidermolysis bullosa is a group of diseases caused by mutations in genes for proteins responsible for cells' anchorage at the dermo-epidermal junction. Their common feature are dysfunctional or even absent connections between cells. The typical clinical sign is the formation of blisters, with possible excessive scarring, in response to minimal skin irritation. End stage renal disease may be one of the comorbidities in patients with epidermolysis bullosa. The implementation of renal replacement therapy may be very difficult in this population...
June 14, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28611842/betulin-based-oleogel-to-improve-wound-healing-in-dystrophic-epidermolysis-bullosa-a-prospective-controlled-proof-of-concept-study
#18
Agnes Schwieger-Briel, Dimitra Kiritsi, Christoph Schempp, Cristina Has, Hauke Schumann
INTRODUCTION: Skin fragility and recurrent wounds are hallmarks of hereditary epidermolysis bullosa (EB). Treatment options to accelerate wound healing are urgently needed. Oleogel-S10 contains a betulin-rich triterpene extract from birch bark. In this study, we tested the wound healing properties of topical Oleogel-S10 in patients with dystrophic EB. METHODS: We conducted an open, blindly evaluated, controlled, prospective phase II pilot trial in patients with dystrophic EB (EudraCT number 2010-019945-24)...
2017: Dermatology Research and Practice
https://www.readbyqxmd.com/read/28599695/junctional-epidermolysis-bullosa-non-herlitz-type
#19
Munir Ahmad Bhinder, Muhammad Waqar Arshad, Muhammad Yasir Zahoor, Wasim Shehzad, Muhammad Tariq, Muhammad Imran Shabbir
Junctional epidermolysis bullosa (JEB) is a recessively inherited skin blistering disease and is caused due to abnormalities in proteins that hold layers of the skin. Herlitz JEB is the severe form and non-Herlitz JEB is the milder form. This report describes a case of congenitally affected male child aged 5 years, with skin blistering. He has mitten-like hands and soft skin blistering on hands, legs and knees. Symptoms almost disappeared at the age of 3 years but reappeared with increased severity after 6 months...
May 2017: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/28592171/next-generation-human-skin-constructs-as-advanced-tools-for-drug-development
#20
H E Abaci, Zongyou Guo, Yanne Doucet, Joanna Jacków, Angela Christiano
Many diseases, as well as side effects of drugs, manifest themselves through skin symptoms. Skin is a complex tissue that hosts various specialized cell types and performs many roles including physical barrier, immune and sensory functions. Therefore, modeling skin in vitro presents technical challenges for tissue engineering. Since the first attempts at engineering human epidermis in 1970s, there has been a growing interest in generating full-thickness skin constructs mimicking physiological functions by incorporating various skin components, such as vasculature and melanocytes for pigmentation...
January 1, 2017: Experimental Biology and Medicine
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