 |
 |

19-DEJ-1, a Monoclonal Antibody to the Hemidesmosome-Anchoring Filament Complex, Is the Only Reliable Immunohistochemical Probe for All Major Forms of Junctional Epidermolysis Bullosa
Jo-David Fine, MD
Arch Dermatol. 1990;126(9):1187-1190.
Abstract
 |  |
Recently, a monocional antibody, 19-DEJ-1, has been described with binding specificity for an epitope present within the mid-lamina lucida of the dermoepidermal junction directly underneath hemidesmosomes, suggesting recognition of a portion of the anchoring filaments. In an initial survey of specimens from patients with inherited epidermolysis bullosa (EB), it was noted that 9 of 9 specimens of Herlitz junctional EB and approximately 50% of specimens of recessive dystrophic EB lacked staining of the dermoepidermal junction with this antibody. To better define the sensitivity of binding by 19-DEJ-1 in junctional EB skin, 40 consecutive specimens representing the three major subtypes (Herlitz, 14; non-Herlitz, 15; and indeterminate, 11) were examined. No staining was noted along the dermoepidermal junction in any specimen, regardless of junctional EB subtype. Considering the recently discovered variability of binding by GB3 monoclonal antibody in some junctional EB subtypes, based on the present data it appears that the 19-DEJ-1 monoclonal antibody is the only immunohistochemical probe that can be used reliably for diagnosis of all major forms of junctional EB. Furthermore, these data suggest the possible utility of this particular antibody as a probe for identification, at the molecular level, of a basement membrane defect shared among all major forms of junctional EB.
(Arch Dermatol. 1990;126:1187-1190)
Author Affiliations
From the Department of Dermatology, University of Alabama School of Medicine, Birmingham.
Footnotes
Accepted for publication March 6, 1990.
Reprint requests to the Department of Dermatology, University of North Carolina at Chapel Hill, Room 137, North Carolina Memorial Hospital, Chapel Hill, NC 27514 (Dr Fine).
CiteULike Connotea Del.icio.us Digg Reddit Technorati Twitter
What's this?
THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES
Prenatal Diagnosis of Genetic Skin Disease Using Fetal Skin Biopsy Samples
Holbrook et al.
Arch Dermatol 1993;129:1437-1454.
ABSTRACT
The Pyloric Atresia--Junctional Epidermolysis Bullosa Syndrome: Report of a Case and Review of the Literature
Lestringant et al.
Arch Dermatol 1992;128:1083-1086.
ABSTRACT
Type VII Collagen and 19-DEJ-1 Antigen: Comparison of Expression in Inversa and Generalized Variants of Recessive Dystrophic Epidermolysis Bullosa
Fine et al.
Arch Dermatol 1990;126:1587-1593.
ABSTRACT
|