Immunopathogenesis of pemphigus vulgaris: A brief review

Abstract

Pemphigus vulgaris is an autoimmune blistering disease of the skin and mucous membrane. It is mediated by autoantibody against desmoglein 3, an intracellular adhesion glycoprotein that forms a part of desmosome. Loss of tolerance to this autoantigen happens for a still unknown reason. However, this disease has a very strong association with some human leukocyte antigen molecules whose structure favors the binding of certain peptide of desmoglein 3. The mechanism of the action of autoantibody has turned out to be more complicated than it was thought, and it is not well established yet. Nevertheless, in the past few years, important discoveries have been made that point to a role of autoantibodies in staining different signaling cascades that lead to loss of adhesion between keratinocyte and death of these cells resulting in acantholysis as histologic manifestation and blister as clinical manifestation.

Pemphigus patients with initial negative levels of anti‐desmoglein: A subtype with different profile?

“…IgG1 seems to be the most sensitive indicator for activity of the disease 5 . These autoantibodies culminate in interrogation of the cell–cell adhesion of keratinocyte in the epidermis, an event called acantholysis 6 . Furthermore, development of blister in PV patients might stem from dysregulated apoptosis, aberrant regulation of cell signaling, higher levels of pro‐inflammatory cytokines, and stimulation of muscarinic receptors on the keratinocytes 7,8 .…”

Evaluating the risk‐to‐benefit ratio of using cotrimoxazole as a pneumocystis pneumonia preventative intervention among pemphigus patients treated with rituximab: A retrospective study with 494 patients

“…The etiopathogenesis of pemphigus is classically attributed to autoantibodies targeting two critical desmosomal proteins, desmoglein (Dsg) 1 and Dsg3, which leads to intraepithelial deposition of autoantibodies. These events result in skin damage by immune hyperresponsiveness that is commonly involved in the development of pemphigus vulgaris (PV) 3 . In terms of clinicopathological presentations, pemphigus is categorized into several subtypes, including PV, pemphigus foliaceus (PF), pemphigus vegetans, paraneoplastic pemphigus, drug‐induced pemphigus, and pemphigus erythematosus 4 …”

“…These events result in skin damage by immune hyperresponsiveness that is commonly involved in the development of pemphigus vulgaris (PV). 3 In terms of clinicopathological presentations, pemphigus is categorized into several subtypes, including PV, pemphigus foliaceus (PF), pemphigus vegetans, paraneoplastic pemphigus, drug-induced pemphigus, and pemphigus erythematosus. 4 Same as many other autoimmune diseases, no definitive cure is available for pemphigus patients and currently available medications can only control the disease and treat active pemphigus-related lesions.…”

Pemphigus Vulgaris Combination Adjuvant Therapy of Systemic Corticosteroid with Sparing Agent Sodium Mycophenolate

“…4 Based on medical record data of out patient and inpatient fromdermatology and venereology department in RSMH Palembang, there were 63 cases of PV in 2018-2019, 41 are male, the other is women Four theories are explaining the pathogenesis of PV, such as desmoglein compensation theory, multiple hits hypothesis, apoptosis induced by antibodies and basal cell shrinkage hypothesis, and apoptosistheory. 5 Major clinical finding in PV are lesions on skin and mucosa.…”