19-DEJ-1, a hemidesmosome-anchoring filament complex-associated monoclonal antibody. Definition of a new skin basement membrane antigenic defect in junctional and dystrophic epidermolysis bullosa

Alpha‐fetoprotein and acetylcholinesterase are not predictive of fetal junctional epidermolysis bullosa, Herlitz variant

“…Diagnosis of fetuses or newborns affected with junctional EB-Herlitz can be made by several techniques, all currently requiring skin biopsies. The diagnosis is made by electron microscopic visualization of blisters within the lamina iucida and observation of reduced numbers and hypoplastic structure of hemidesmosomes (Hashimoto et al, 1976;Anton-Lamprecht 198 1) or by immunofluorescent demonstration of normal or altered binding of two monoclonal antibodies (i.e., GB3 and 19-DEJ-1) to the skin basement membrane zone (Fine, 1990;Fine et al, 1989Fine et al, ,1990Heagerty et at., 1986aHeagerty et at., , b, 1987Schofield et al, 1990). As such, prenatal diagnosis of this disorder requires obtaining fetal skin biopsies, initially performed by fetoscopy but now most commonly performed by ultrasound-guided fetal skin biopsy (Elias and Easterly, 1981;Bakharev c't al., 1990;Shulman and Elias, 1990).…”

“…Of note is that prior to insertion of biopsy forceps, 310 ml of amniotic fluid were aspirated for cytogenetic and AFP analysis. Four to six fetal skin biopsies were then obtained and analysed by light and electron microscopy as well as immunohistochemical studies to include immunofluorescent antigenic mapping and EB-specific (GB3 and 19-DEJ-1) monoclonal antibody studies (Fine, 1990;Fine et al, 1989;Heagerty et al, 1986aHeagerty et al, , 1987Schofield et al, 1990).…”

Prenatal Diagnosis of Epidermolysis bullosa: First Successful Trial in Asia

“…Prenatal Diagnosis of Epidermolysis bullosa DEJ-1 [17,18], LH7.2 [13][14][15], has made the prenatal diag nosis of EB more reliable and faster [6,7], For example, absence of staining with anti-type-VII-collagen-associated antibody (LH7.2) would be most consistent with the find ings made exclusively in severe generalized RDEB. Simi larly, absence of staining with GB3 is consistent with those made in gravis JEB.…”

Successful prenatal exclusion of an unspecified subtype of severe epidermolysis bullosa

“…Cryostat sections of unfixed tissue, each of 6 µm, were subjected to indirect immunofluorescence. Sections were incubated for 60 min at 37°C with a panel of primary antibodies as shown in Table 1, including LH7.2, [17][18][19] GB3, 20 19-DEJ-1 MoAb, 21,22 GoH3, 3E1, D20, 23 S1193, and 121. 24 MoAb against human type IV collagen and bullous pemphigoid sera against the 230-kDa BPAG1 25,26 were used as controls.…”