Pemphigus-The Crux of Clinics, Research, and Treatment during the COVID-19 Pandemic

被引:5
作者
Marinovic, Branka [1 ]
Mise, Josko [2 ]
Jukic, Ines Lakos [1 ]
Bukvic Mokos, Zrinka [1 ]
机构
[1] Univ Zagreb, Univ Hosp Ctr Zagreb, Sch Med, Dept Dermatol & Venereol, Salata 4, Zagreb 10000, Croatia
[2] Univ Hosp Ctr Zagreb, Dept Dermatol & Venereol, European Reference Network ERN Skin Reference Ctr, Kispaticeva 12, Zagreb 10000, Croatia
关键词
pemphigus; desmoglein; rituximab; immunodermatology; COVID-19; SARS-CoV-2; IGG/IGA PEMPHIGUS; CLINICOPATHOLOGICAL FEATURES; VULGARIS; RITUXIMAB; DISEASES; ANTIBODIES; MANAGEMENT; THERAPY; MODIFY; IGG;
D O I
10.3390/biomedicines9111555
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
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.
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页数:14
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