Abstract
Pemphigus is a rare autoimmune disease characterised by the production of pathogenic autoantibodies in response to different desmosome proteins. The pathophysiological process leads to the development of blisters and erosions on mucosal and/or skin surfaces. The classical clinical variants of pemphigus are pemphigus vulgaris and pemphigus foliaceus. A diagnostic delay is very common in pemphigus, especially among patients with mucosal involvement. However, in recent years we have witnessed considerably fewer patients with extensive mucocutaneous manifestations, since patients with oral lesions are referred to dermatologists to start the treatment much sooner than they had been previously. Among non-classical variants of pemphigus, unusual cases with discrepancies between autoantibody profiles and clinics challenge the “desmoglein compensation theory”. The identification of several other autoantigens that perform a role in the pathogenesis of different variants of pemphigus will progress immunodermatology towards an approach that will determine personalized pemphigus subtypes for each patient. Comorbidities among patients are primarily associated with the prolonged use of corticosteroids and other immunosuppressive agents. The SARS-CoV-2 pandemic raised concerns regarding the immunosuppressive effects of treatment and the risk of a more complicated COVID-19 infection, as well as on the ability to develop an adequate vaccine response.
Highlights
Pemphigus diseases are a group of rare autoimmune bullous diseases that affect the skin and mucous membranes
The results have revolutionised the management of pemphigus vulgaris (PV) as there are many studies published in recent years suggesting that rituximab is an effective agent in inducing remission when used as the first-line treatment; the reported rituximab efficiency in achieving complete remission ranges from 58% to over 90% of the patients included in the observed studies [20,21,22,23,24,25]
Pemphigus vegetans is a rare form of pemphigus vulgaris, accounting for 1–2% of all cases of pemphigus [27]
Summary
Pemphigus diseases are a group of rare autoimmune bullous diseases that affect the skin and mucous membranes. They are immunopathologically characterised by the production of pathogenic autoantibodies that are directed against different proteins of desmosomes, leading to acantholysis and the formation of vesicles, blisters, and erosions on the skin and/or mucous membranes. Desmoglein 1 (Dsg 1) and desmoglein 3 (Dsg 3) are the primary target antigens in pemphigus. They belong to the cadherin gene family of Ca2+. Different types of pemphigus have been identified based on the clinical and histopathological features, as well as on the specific antigens against which the autoantibodies are produced. PV is the most common clinical form of pemphigus, accounting for approximately 70% of cases; it is considered the most severe form of the disease [4]
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