Potential of Fibroblast Cell Therapy for Recessive Dystrophic Epidermolysis Bullosa

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Potential of fibroblast cell therapy for recessive dystrophic epidermolysis bullosa.

Recessive dystrophic epidermolysis bullosa (RDEB) is a severe inherited skin-blistering disorder caused by mutations in the COL7A1 gene that lead to reduced type-VII collagen and defective anchoring fibrils at the dermal-epidermal junction (DEJ). Presently there are no effective treatments for this disorder. Recent mouse studies have shown that intradermal injections of normal human fibroblasts...

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Dystrophic epidermolysis bullosa (DEB) is a severe skin fragility disorder associated with trauma-induced blistering, progressive soft tissue scarring, and increased risk of skin cancer. DEB is caused by mutations in the COL7A1 gene which result in reduced, truncated, or absent type VII collagen, and anchoring fibrils at the dermal-epidermal junction (DEJ). Because no topical wound-healing agen...

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Three Brothers with Recessive Dystrophic Epidermolysis Bullosa

Epidermolysis bullosa (EB) is a group of heritable mechano-bullous skin diseases classified into three major categories on the basis of the level of tissue separation within the dermal-epidermal basement membrane zone. In the most severe, dystrophic (scarring) forms of EB, blisters form below the cutaneous basement membrane at the level of the anchoring fibrils, which are composed of type VII c...

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application of allogeneic fibroblast cells in cellular therapy of recessive dystrophic epidermolysis bullosa

context connective tissue cells include fibroblasts, chondrocytes, adipocyte, and osteocytes. these cells are specialized for the secretion of collagenous extracellular matrix and are responsible for the architectural framework of the human body. evidence acquisition connective tissue cells play a central role in supporting as well as repairing tissues and organs. fibroblast cell therapy could ...

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A 22-year-old male with recessive dystrophic epidermolysis bullosa with a large superficial and nodular basal cell carcinoma on his right forehead was treated with photodynamic therapy. The treatment was well tolerated, and the site healed well. Patients with epidermolysis bullosa are at increased risk of developing skin cancers, particularly squamous cell carcinomas. However, basal cell carcin...

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ژورنال

عنوان ژورنال: Journal of Investigative Dermatology

سال: 2008

ISSN: 0022-202X

DOI: 10.1038/jid.2008.78