2 Human keratinocytes form a correctly differentiated epidermis with an in Wvo-like dermal-epidermal junction
24,25) Usually, there is evidence of interface dermatitis, including vacuolar alteration of basal keratinocytes, dyskeratotic keratinocytes, and blurring of the dermal-epidermal junction
Meticulous wound care and immobilisation are critical as EB patients are predisposed to delayed wound healing (from a deficiency of Laminin-5, a keratinocyte adhesion factor) and surgical site infections (SSIs) (from accumulation of serum in the dermal-epidermal junction
and impaired lymphocyte function) (3,5,6).
Specialized cells found near the dermal-epidermal junction
, characterized by numerous membrane-bound granules with dense cores.
It is hypothesized that binding of autoantibodies to BP180 initiates an inflammatory reaction, leading to blister formation at the dermal-epidermal junction
The acquired type (epidermolysis bullosa acquisita) is a chronic autoimmune disorder characterized by the presence of autoantibodies against type VII collagen, which make up the anchoring fibrils along the dermal-epidermal junction
Another common presentation is stellate or hypopigmented scars from torsional stress in photodamaged skin with a weak dermal-epidermal junction
Laminin also accelerates the assembly of basement membranes at the dermal-epidermal junction
and its deposition can instruct keratinocytes to switch from an activated phenotype to a quiescent and integrated epithelial phenotype (Nguyen et al.
Dystrophic epidermolysis bullosa (EB) can have an extremely variable progression that depends, in part, on the underlying molecular defects in many different genes expressed in the dermal-epidermal junction
, according to study investigators Hassan Vahidnezhad, MD, of the department of dermatology and cutaneous biology, Thomas Jefferson University, Philadelphia, and his associates.
The partnership's preliminary results using FCX-007 cells in a human skin graft model showed no findings of toxicology in RDEB human skin xenograft severe combined immunodeficiency (SCID) mice, additional positive proof-of-concept data from in vivo pre-clinical studies showed the presence of COL7 in the dermal-epidermal junction
of the RDEB cultured grafts in RDEB human skin xenograft SCID mice and found no systemic distribution of the vector in normal human skin xenograft SCID mice.