It is characterised by autoantibodies against desmoglein-3
. It shows a geographical predilection-Mediterranean, South Asia and Jewish region are affected more.
Other negative markers include Desmoglein-3
and Connexin 43 (Cx43) and Connexin 50 (Cx50), both expressed in the corneal epithelium and absent in the basal limbal epithelium [23, 33-35].
Patients affected by pemphigus have immune globulin G autoantibody against desmosomal components like desmoglein-1 and desmoglein-3
Histologically, the disease is characterized primarily by the presence of IgG4 antibodies against desmoglein-3
, a cadherin-like transmembrane glycoprotein that mediates cell-cell adhesion in keratinocytes of the epidermis and mucous membranes.
However, in the superficial layers of the skin it is a major protein responsible for cell-to-cell attachment, whereas in the deep epidermis other adhesion molecules are coexpressed, such as desmoglein-3
and Ecadherin, both resistant to lysis by S.
Lung Adenocarcinoma Versus Lung Squamous Cell Carcinoma (Second IHC Panel) Lung Lung Squamous Marker Adenocarcinoma Cell Carcinoma CK7 + -/+ p63 -/+ + SOX2 - + Desmoglein-3
- + Abbreviations: CK7, cytokeratin 7; IHC, immunohistochemical; SOX2, sex-determining region Y box 2; +, greater than 75% of cases are positive; -, fewer than 5% of cases are positive; -/+, fewer than 50% of cases are positive.
In pemphigus, blistering of the mouth precedes blistering of the skin, and blisters in the mouth are associated with the presence of antibodies against desmoglein-3
protein, which is specific to the mouth epithelial cell antigens.
Less studied, emerging therapies include intravenous immunoglobulin, plasmapheresis, immunoadsorption, extracorporeal photochemotherapy, cholinergic agonists, rituximab, anti-CD20 monoclonal antibody therapy, tumor necrosis factor-[alpha] (TNF-[alpha]) inhibitors and other experimental therapies such as Desmoglein-3
TTF-1, Napsin A, p63, TRIM 29, Desmoglein-3
and CK5: An Evaluation of Sensitivity and Specificity, and Correlation of Tumor Grade for Lung Squamous Cell Carcinoma vs.
The emergence of novel and better squamous cell carcinoma (SCC) markers, such as p40 and desmoglein-3
, coupled with double staining, enhances the possibility of effectively separating adenocarcinoma (ADC) from SCC on small biopsies and cytologic specimens, conserving tissue for molecular testing.
Lopez et al., "Development and characterization of desmoglein-3
specific T cells from patients with pemphigus vulgaris," The Journal of Clinical Investigation, vol.