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Oral Manifestations and Challenges in Dental Treatment of Epidermolysis Bullosa Dystrophica

Source: Journal of Dentistry for Children - Category: Dentistry Authors: Tags: Case Reports Source Type: research

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Epidermolysis bullosa simplex with muscular dystrophy (EBS-MD; OMIM #226670) is an autosomal recessive disease, characterized mainly by skin blistering at birth or shortly thereafter, progressive muscle weakne...
Source: BMC Dermatology - Category: Dermatology Authors: Tags: Case report Source Type: research
Abstract Type VII collagen (COL7), a major component of anchoring fibrils in the epidermal basement membrane zone, has been characterized as a defective protein in dystrophic epidermolysis bullosa and as an autoantigen in epidermolysis bullosa acquisita. Although COL7 is produced and secreted by both epidermal keratinocytes and dermal fibroblasts, the role of COL7 with regard to the epidermis is rarely discussed. This review focuses on COL7 physiology and pathology as it pertains to epidermal keratinocytes. We summarize the current knowledge of COL7 production and trafficking, its involvement in keratinocyte dynamics, and ...
Source: The Journal of Dermatology - Category: Dermatology Authors: Tags: Review Article Source Type: research
CONCLUSION: iscorEB has robust reliability and construct validity, including strong ability to distinguish EB types and severities. Further studies are planned to test its responsiveness to change. This article is protected by copyright. All rights reserved. PMID: 29341069 [PubMed - as supplied by publisher]
Source: The British Journal of Dermatology - Category: Dermatology Authors: Tags: Br J Dermatol Source Type: research
ConclusionsEBSEQ is a sensitive, relatively rapid, minimally invasive, comprehensive genetic assay for the diagnosis of epidermolysis bullosa.
Source: Pediatric Dermatology - Category: Dermatology Authors: Tags: Original Article Source Type: research
Source: International Journal of Dermatology - Category: Dermatology Authors: Tags: Correspondence Source Type: research
Abstract BACKGROUND: Different methods of fibroblast application have been examined to treat recessive dystrophic epidermolysis bullosa (RDEB). OBJECTIVE: To compare the effects of intradermal injection of cultured allogeneic fibroblasts in healing RDEB wounds with that of fibroblasts seeded on amniotic membrane scaffolds (FAMS) or standard wound care (SWC) with Vaseline gauze as controls. MATERIALS &METHODS: Seven patients were recruited, and seven wounds were assessed in each patient: three wounds were treated with injection of intradermal fibroblasts, three were treated with FAMS, and one was dressed ...
Source: The British Journal of Dermatology - Category: Dermatology Authors: Tags: Br J Dermatol Source Type: research
ngjel J Abstract The extracellular matrix protein collagen VII is part of the microenvironment of stratified epithelia and critical in organismal homeostasis. Mutations in the encoding gene COL7A1 lead to the skin disorder dystrophic epidermolysis bullosa (DEB), are linked to skin fragility and progressive inflammation-driven fibrosis that facilitates aggressive skin cancer. So far, these changes have been linked to mesenchymal alterations, the epithelial consequences of collagen VII loss remaining under-addressed. As epithelial dysfunction is a principal initiator of fibrosis, we performed a comprehensive transcr...
Source: Molecular and Cellular Proteomics : MCP - Category: Molecular Biology Authors: Tags: Mol Cell Proteomics Source Type: research
Epidermolysis bullosa acquisita (EBA) is a rare mucocutaneous blistering disorder with typical onset in adulthood. Diagnosis and management can be difficult owing to the variability in presentation and clinical manifestation. In this case, we explore a case of EBA as well as provide a general overview of the condition and its variants.Dermatopathology 2018;5:1-5
Source: Dermatopathology - Category: Pathology Source Type: research
Source: British Journal of Dermatology - Category: Dermatology Authors: Tags: Letter to the Editor Source Type: research
PMID: 29322498 [PubMed - as supplied by publisher]
Source: The British Journal of Dermatology - Category: Dermatology Authors: Tags: Br J Dermatol Source Type: research
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