Summary
The very late antigen (VLA) glycoproteins are a family of adhesion membrane receptors involved in cell-cell and cell-matrix interactions. In order to investigate the expression of these molecules in inherited epidermolysis bullosa (EB), we studied the reactivity of monoclonal antibodies directed against VLA-1, -2, -3, -4, -5, and -6, and VLAΒ receptors in skin sections from patients affected by several types of EB simplex (EBs) using indirect immunofluorescence. Skin samples were obtained from six patients with generalized type (Koebner), one patient with localized type (Weber-Cockayne) and one patient with Dowling-Meara EBs type and also from two normal controls. No significant modification of the expression of these adhesion receptors was observed. Anti-VLA-2 and anti-VLA-3 stained the whole cytoplasmic membrane of basal keratinocytes and allowed the detection of focal areas of cytolysis in unblistered skin from the Koebner and Dowling-Meara type. In Koebner type blisters anti-VLA-3 stained the cell remnants at the roof of the blister with a linear staining along the epidermal basement membrane on the dermal side. In Dowling-Meara type blisters anti-VLA-3 also stained cell remnants at the bottom of the cavity. Anti-VLA-6 stained the bottom of the blister cavity with the same distribution of bullous pemphigoid serum but with a stronger and more constant reactivity. Our data show that anti-VLA-3 and anti-VLA-6 can usefully be utilized in diagnostic immunomapping studies of EBs.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Anton-Lamprecht I, Schnyder UW (1982) Epidermolysis bullosa herpetiformis Dowling-Meara. Report of a case and pathomorphogenesis. Dermatologica 164:221–235
Buck CA, Horwitz AF (1987) Integrin, a transmembrane glycoprotein complex mediating cell-substratum adhesion. J Cell Sci [Suppl 8]:231–250
Fine JD (1987) Altered skin basement membrane antigenicity in epidermolysis bullosa. Curr Probl Dermatol 17:111–126
Fine JD (1988) Antigenic features and structural correlates of basement membranes. Relationship to epidermolysis bullosa. Arch Dermatol 124:713–717
Fine JD, Horiguchi Y, Couchman JR (1989) 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. Arch Dermatol 125:520–523
Gedde-Dahl T Jr (1981) Sixteen types of epidermolysis bullosa. On the clinical discrimination, therapy, and prenatal diagnosis. Acta Derm Venereol Suppl (Stockh) 95:74–87
Gedde-Dahl T Jr, Anton-Lamprecht I (1983) Epidermolysis bullosa. In: Emery AEH, Rimoin DL (eds) Principles and practice of medical genetics. Churchill Livingstone, Edinburgh, pp 672–687
Heagerty AH, Kennedy AR, Eady RA, Hsi BL, Verrando P, Yeh CJ, Ortonne JP (1986) GB3 monoclonal antibody for diagnosis of junctional epidermolysis bullosa. Lancet I:860
Heagerty AH, Kennedy AR, Leigh IM, Purkis P, Eady RA (1986) Identification of an epidermal basement membrane defect in recessive forms of dystrophic epidermolysis bullosa by LH 7∶2 monoclonal antibody:nuse in diagnosis. Br J Dermatol 115:125–131
Hemler ME (1988) Adhesive protein receptors on hematopoietic cells. Immunol Today 9:109–113
Hemler ME, Huang C, Schwarz L (1987) The VLA protein family. Characterization of five distinct cell surface heterodimers each with a common 130000 molecular weightΒ subunit. J Biol Chem 262:3300–3309
Hintner H, Stingl G, Schuler G, Fritsch P, Stanely J, Katz S, Wolff K (1981) Immunofluorescence mapping of antigenic determinants within the dermal-epidermal junction in mechanobullous diseases. J Invest Dermatol 76:113–118
Hynes RO (1987) Integrins: a family of cell surface receptors. Cell 48:549–554
Kaufmann R, Frosch D, Westphal C, Weber L, Klein CE (1989) Integrin VLA-3: ultrastructural localization at cell-cell contact sites of human cell cultures. J Cell Biol 109:1807–1815
Kitajima Y, Inoue S, Yaoita H (1989) Abnormal organization of keratin intermediate filaments in cultured keratinocytes of epidermolysis bullosa simplex. Arch Dermatol Res 281:5–10
Knapp W, Dörken B, Gilks WR, Rieber EP, Schmidt RE, Stein H, Borne AEGKr von dem (eds) (1989) Leukocyte typing IV, white cell differentiation antigens. Oxford University Press, Oxford New York Tokyo, pp 1017–1036
Konter U, Kellner I, Klein E, Kaufmann R, Mielke V, Sterry W (1989) Adhesion molecule mapping in normal human skin. Arch Dermatol Res 281:454–462
Nazzaro V, Berti E, Cerri A, Brusasco A, Cavalli R, Caputo R (1990) Expression of integrins in junctional and dystrophic epidermolysis bullosa. J Invest Dermatol 95:60–64
Pischel KD, Hemler ME, Huang C, Bluestein HG, Woods VL Jr (1987) Use of the monoclonal antibody 12F1 to characterize the differentiation antigen VLA-2. J Immunol 138:226–233
Sánchez-Madrid F, De Landázuri MO, Morago G, Cebrián M, Acevedo A, Bernabeu C (1986) VLA-3: a novel polypeptide association within the VLA molecular complex: cell distribution and biochemical characterization. Eur J Immunol 16:1343–1349
Sonnenberg A, Modderman PW, Hogervorst F (1988) Laminin receptor on platelets is the integrin VLA-6. Nature 336:487–489
Wayner EA, Garcia-Pardo A, Humphries MJ, McDonald JA, Carter WG (1989) Identification and characterization of the T lymphocyte adhesion receptor for an alternative cell attachment domain (CS-1) in plasma fibronectin. J Cell Biol 109:1321–1330
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Nazzaro, V., Berti, E., Cavalli, R. et al. Very late antigen (VLA) expression in various forms of epidermolysis bullosa simplex. Arch Dermatol Res 283, 1–4 (1991). https://doi.org/10.1007/BF01207240
Received:
Issue Date:
DOI: https://doi.org/10.1007/BF01207240