Book Chapter10.1007/978-1-59259-726-0_88
Pemphigus Foliaceus and Pemphigus Vulgaris
Janet A. Fairley,Xiang Ding,George J. Giudice,Luis A. Diaz +3 more
- 01 Jan 1998
- pp 811-816
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TL;DR: Pemphigus is a group of autoimmune disorders characterized by spontaneous intraepidermal blisters and epidermal-specific autoantibodies and there is an endemic form of PF that has been observed in Brazil since the turn of the century and recently in Colombia.
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Abstract: Pemphigus is a group of autoimmune disorders characterized by spontaneous intraepidermal blisters and epidermal-specific autoantibodies. Blister formation occurs by the histologically distinctive loss of epidermal cell—cell adhesion, termed acantholysis. The two major forms of the disease are pemphigus vulgaris (PV) and pemphigus foliaceus (PF). In PV, the epidermal-cell separation occurs just above the basal layer of the epidermis, whereas, in PF, the cell separation occurs at the more superficial level of the granular-cell layer of the epidermis. Although PV and PF are diseases described all over the world, there is an endemic form of PF that has been observed in Brazil since the turn of the century and recently in Colombia. This endemic form of PF is also known as “fogo selvagem” (FS; wild fire). Prior to the advent of immunosuppressive therapy, the mortality caused by these disorders was extremely high.
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Citations
Desmoglein 3-ELISA: a pemphigus vulgaris-specific diagnostic tool.
TL;DR: The Dsg3-ELISA is a sensitive, objective, and PV-specific test that should be considered as an adjunct test for the management of patients with PV.
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The Enigmatic Autoimmune Response in Endemic Pemphigus Foliaceus
Gustavo Flores,Ye Qian,Luis A. Diaz +2 more
- 01 Dec 2009
TL;DR: It is hypothesized that the pathogenic IgG4 response in FS may be triggered by hematophagous insect bites, and the literature on the relevance of the humoral autoimmune response of FS as well as the possible environmental triggers of anti-Dsg1 autoantibody formation is reviewed.
References
Autoantibodies against a novel epithelial cadherin in pemphigus vulgaris, a disease of cell adhesion
TL;DR: It is demonstrated that a novel epithelial cadherin is the target of autoantibodies in PV, and the deduced amino acid sequence of PVA was unique but showed significant homology with members of the cadher in family of Ca(2+)-dependent cell adhesion molecules.
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Induction of Pemphigus in Neonatal Mice by Passive Transfer of IgG from Patients with the Disease
TL;DR: This study strongly supports the proposed role of pemphigus autoantibodies in the pathogenesis of p emphigu vulgaris in human beings and demonstrates that pemPHigus can be passively transferred to laboratory animals.
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Targeted Disruption of the Pemphigus Vulgaris Antigen (Desmoglein 3) Gene in Mice Causes Loss of Keratinocyte Cell Adhesion with a Phenotype Similar to Pemphigus Vulgaris
Peter Koch,My G. Mahoney,Hiroyasu Ishikawa,Leena Pulkkinen,Jouni Uitto,Leonard Shultz,George F. Murphy,Diana Whitaker-Menezes,John R. Stanley +8 more
TL;DR: It is suggested that pemphigus autoantibodies might interfere directly with such a function of DSG3, and the critical importance of Dsg3 for adhesion in deep stratified squamous epithelia is demonstrated.
Demonstration of an adhering-junction molecule (plakoglobin) in the autoantigens of pemphigus foliaceus and pemphigus vulgaris.
TL;DR: It is concluded that plakoglobin, a protein of the adhering junctions of epidermal cells, is the 85-kd molecule in the antigenic complexes found in both pemphigus foliaceus and pemPHigus vulgaris, although it is not the binding site in either disorder.
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Antigen-specific immunoadsorption of pathogenic autoantibodies in pemphigus foliaceus.
Masayuki Amagai,Masayuki Amagai,Takashi Hashimoto,Kathleen J. Green,Nobuyoshi Shimizu,Takeji Nishikawa +5 more
TL;DR: Observations indicate that the extracellular domain of Dsg1 expressed by baculovirus is capable of specifically immunoadsorbing pathogenic autoantibodies from PF patients' sera and provide direct evidence that the anti-Dsg1 autoantsibodies in PF sera are indeed pathogenic.
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