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In vitro organ culture model for mucous membrane pemphigoid.
- Source :
-
Clinical immunology (Orlando, Fla.) [Clin Immunol] 2001 Feb; Vol. 98 (2), pp. 229-34. - Publication Year :
- 2001
-
Abstract
- Mucous membrane pemphigoid (MMP), also known as cicatricial pemphigoid, is a rare vesiculobullous disease of mucosal tissues, which involves the oral, ocular, and other mucous membranes. We have studied a group of patients with histologically and immunopathologically proven pemphigoid disease involving predominantly the conjunctiva and oral mucosa in addition to other mucosae. The purpose of our study was to (i) demonstrate the specific binding of autoantibodies present in the sera of patients with MMP to normal human oral mucosa by indirect immunofluorescence (IIF) and (ii) to study the role of these autoantibodies in the pathogenesis of subepithelial blister formation using normal human buccal mucosa in organ culture. Serum and IgG fractions from MMP patients showed homogeneous smooth linear binding along the basement membrane zone (BMZ) of the normal buccal mucosa on IIF. Serum from pemphigus vulgaris patients showed intercellular or keratinocyte cell surface staining. BMZ separation developed at 48 h after incubation of normal human buccal mucosa in organ culture, with serum or IgG from patients with MMP but not after addition of normal human serum. Addition of pemphigus vulgaris serum to the in vitro culture of normal human buccal mucosa showed acantholysis. This preliminary report suggests that circulating autoantibodies may have an important role in the pathogenesis of MMP. This in vitro organ culture model will facilitate enhancing our understanding of various molecular events during the process of blister formation in MMP and in the study of other mucosal diseases.<br /> (Copyright 2000 Academic Press.)
- Subjects :
- Acantholysis
Animals
Autoantibodies blood
Autoimmune Diseases blood
Autoimmune Diseases immunology
Basement Membrane immunology
Cattle
Conjunctiva immunology
Fluorescent Antibody Technique, Indirect
Humans
Immunoglobulin G blood
Immunoglobulin G immunology
Integrin beta4
Keratinocytes immunology
Mouth Mucosa immunology
Pemphigoid, Bullous blood
Pemphigoid, Bullous immunology
Pemphigus blood
Pemphigus immunology
Antigens, CD immunology
Autoantibodies immunology
Autoantigens immunology
Autoimmune Diseases pathology
Conjunctiva pathology
Mouth Mucosa pathology
Organ Culture Techniques methods
Pemphigoid, Bullous pathology
Subjects
Details
- Language :
- English
- ISSN :
- 1521-6616
- Volume :
- 98
- Issue :
- 2
- Database :
- MEDLINE
- Journal :
- Clinical immunology (Orlando, Fla.)
- Publication Type :
- Academic Journal
- Accession number :
- 11161979
- Full Text :
- https://doi.org/10.1006/clim.2000.4972