New insights into molecular genetics and pathomechanisms in epidermolysis bullosa (EB), methodological and technological advances in molecular biology as well as designated funding initiatives and facilitated approval procedures for orphan drugs have boosted translational research perspectives for this devastating disease. This is echoed by the increasing number of clinical trials assessing innovative molecular therapies in the field of EB. Despite remarkable progress, gene-corrective modalities, aimed at sustained or permanent restoration of functional protein expression, still await broad clinical availability. This also reflects the methodological and technological shortcomings of current strategies, including the translatability of certain methodologies beyond preclinical models as well as the safe, specific, efficient, feasible, sustained and cost-effective delivery of therapeutic/corrective information to target cells. This review gives an updated overview on status, prospects, challenges and limitations of current gene-targeted therapies.
The subcutaneously pedicled flap, also known as V-Y flap, belongs to the group of island flaps whose blood supply (unlike the horizontally perfused advancement and transposition flaps) comes from subcutaneous blood vessels perpendicular to the skin surface. Because of the outstanding perfusion of this type of flap, we remove up to one-third of the cranial flap pedicle to form a skin flap (3-4 mm thick), resulting in a significant increase in the defect size able to be covered by the V-Y flap. Moreover, in the infraorbital region - as far as topographically feasible - this flap is pulled further cranially (in the direction of the defect to be closed) by an obliquely placed periosteal suture. This offsets any potential distal tension on the flap and has thus proven beneficial in preventing an ectropion.
The subcutaneously pedicled flap, also known as V-Y flap, belongs to the group of island flaps whose blood supply (unlike the horizontally perfused advancement and transposition flaps) comes from subcutaneous blood vessels perpendicular to the skin surface. Because of the outstanding perfusion of this type of flap, we remove up to one-third of the cranial flap pedicle to form a skin flap (3-4 mm thick), resulting in a significant increase in the defect size able to be covered by the V-Y flap. Moreover, in the infraorbital region - as far as topographically feasible - this flap is pulled further cranially (in the direction of the defect to be closed) by an obliquely placed periosteal suture. This offsets any potential distal tension on the flap and has thus proven beneficial in preventing an ectropion.
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