| Abstract | Bullous pemphigoid was first described by Lever in 1953 as a subepidermal blistering disease. Its immunohistological features include dermal-epidermal junction separation, an inflammatory cell infiltrate in the upper dermis, and basement membrane zone-bound autoantibodies. These autoantibodies show a linear staining at the dermal-epidermal junction, activate complement, and recognize two major hemidesmosomal antigens, BP230 (BPAG1) and BP180 (BPAG2 or type XVII collagen). An IgG passive transfer mouse model of BP was developed by administering rabbit antimurine BP180 antibodies to neonatal mice. This model recapitulates the key features of human bullous pemphigus. Using this in vivo model system, several key cellular and molecular events leading to the bullous pemphigus disease phenotype were identified, including IgG binding, complement activation, mast cell degranulation, and neutrophil infiltration and activation. Proteinases and reactive oxygen species released by neutrophils work together to damage the basement membrane zone, causing dermal-epidermal junction separation. Recent experimental data from human bullous pemphigus studies suggest that human bullous pemphigus and its mouse IgG passive transfer model counterpart may well share not only common immunohistological features but also pathological mechanisms underlying the development of this antibody-mediated disease. |
| Authors | Zhi Liu
(Affiliation: Department of Dermatology, University of North Carolina, Chapel Hill, North Carolina 27599, USA. zhiliu at med.unc.edu)
|
| Journal | The journal of investigative dermatology. Symposium proceedings / the Society for Investigative Dermatology, Inc. [and] European Society for Dermatological Research
(J Investig Dermatol Symp Proc)
Vol. 9
Issue 1
Pg. 41-6
(Jan 2004)
ISSN: 1087-0024 United States |
| PMID | 14870984
(Publication Type: Journal Article, Research Support, U.S. Gov't, P.H.S., Review)
|
| Chemical References |
- Autoantigens
- Non-Fibrillar Collagens
- collagen type XVII
|
| Topics |
- Amino Acid Sequence
- Animals
- Autoantigens
(genetics, immunology)
- Disease Models, Animal
- Mice
- Molecular Sequence Data
- Non-Fibrillar Collagens
- Pemphigoid, Bullous
(immunology, pathology)
|
