Feasibility, efficacy, and safety of ultrasound‐guided axillary plexus blockade in pediatric patients with epidermolysis bullosa dystrophica

ConclusionUltrasound‐guided plexus axillaris block in DEB patients undergoing hand surgery in our institution has been feasible, effective, and safe.
Source: Pediatric Anesthesia - Category: Anesthesiology Authors: Tags: Original Article Source Type: research

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In this study, using the immunization-induced mouse model of EBA, we demonstrate that epidermal disruption induces not only an infiltration of CD4+ T cells but also a Th1 phenotype as it has been described for delayed-type hypersensitivity reactions.
Source: Journal of Investigative Dermatology - Category: Dermatology Authors: Tags: Original Article Source Type: research
Authors: Bin Nooh DM, Hegazi TM, Bukhari IA Abstract Anonychia refers to the absence of nail plates owing to an autosomal dominant or recessive inheritance. Congenital anonychia is a rare condition that may be associated with other ectodermal or mesodermal malformations like epidermolysis bullosa, (deafness, onychodystrophy, osteodystrophy, and mental retardation) syndrome and Iso-Kikuchi syndrome. Here, we report 3 cases with anonychia congenita appearing in different generations of a single family in Kingdom of Saudi Arabia. PMID: 32020155 [PubMed - in process]
Source: Saudi Medical Journal - Category: Middle East Health Tags: Saudi Med J Source Type: research
This article reviews different approaches to curative and symptom-relief therapies, and appraises their status and perspectives for clinical implementation. PMID: 32039456 [PubMed - as supplied by publisher]
Source: Acta Dermato-Venereologica - Category: Dermatology Authors: Tags: Acta Derm Venereol Source Type: research
Abstract Collagen XVII (COL17) is a hemidesmosomal transmembrane protein in the skin, which, in several autoimmune blistering skin diseases, may be targeted by autoantibodies. In addition, loss-of-function mutations in the COL17A1 gene induce a subtype of junctional epidermolysis bullosa. The extracellular domain of COL17 can be physiologically cleaved from the cell surface by ADAM family proteins in a process known as ectodomain shedding. COL17 ectodomain shedding is thought to be associated with the migration and proliferation of keratinocytes. Furthermore, the C-terminal cleavage of COL17 may be associated with...
Source: Acta Dermato-Venereologica - Category: Dermatology Authors: Tags: Acta Derm Venereol Source Type: research
CONCLUSION: The proposed classification scheme should be of value both to clinicians and researchers, emphasizing both clinical and genetic features of EB. PMID: 32017015 [PubMed - as supplied by publisher]
Source: The British Journal of Dermatology - Category: Dermatology Authors: Tags: Br J Dermatol Source Type: research
CONCLUSIONS: The treatment of edentulous patients with RDEB by means of implants and implant-supported prostheses is predictable as evidenced by the high success rate, and improves patient self-esteem and quality of life. PMID: 31967984 [PubMed - as supplied by publisher]
Source: Medicina Oral, Patologia Oral y Cirugia Bucal - Category: ENT & OMF Tags: Med Oral Patol Oral Cir Bucal Source Type: research
CONCLUSIONS: The selection of the gene therapy method depends on its safety profile, the target genodermatoses and the genetic mutation to correct. PMID: 31965203 [PubMed - as supplied by publisher]
Source: Der Hautarzt: Zeitschrift fur Dermatologie, Venerologie, und verwandte Gebiete - Category: Dermatology Tags: Hautarzt Source Type: research
Epidermolysis bullosa is a rare genetic condition where skin can tear at the slightest touch.
Source: BBC News | Health | UK Edition - Category: Consumer Health News Source Type: news
TO THE EDITOR
Source: Journal of Investigative Dermatology - Category: Dermatology Authors: Tags: Letters to the Editor Source Type: research
Epidermolysis bullosa acquisita (EBA) is an autoimmune skin blistering disease characterized by IgG autoantibodies (aAb) against type VII collagen (COL7). The mechanisms controlling the formation of such aAbs and their effector functions in the skin tissue are incompletely understood. Here, we assessed whether the inhibitory IgG Fc receptor, FcγRIIB, controls the development of autoimmune skin blistering disease in an active model of EBA. For this purpose, we immunized congenic EBA-susceptible B6.SJL-H2s (B6.s) and B6.s-Fcgr2b−/− mice with the immunodominant vWFA2 region of COL7. B6.s-Fcgr2b−/&minus...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
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