Pemphigus vulgaris

Abstract

Pemphigus vulgaris is a chronic autoimmune bullous dermatosis that results from the production of autoantibodies against desmogleins 1 and 3. It is the most frequent and most severe form of pemphigus, occurring universally, usually between 40 and 60 years of age. It usually begins with blisters and erosions on the oral mucosa, followed by lesions on other mucous membranes and flaccid blisters on the skin, which can be disseminated. There is a clinical variant, pemphigus vegetans, which is characterized by the presence of vegetating lesions in the large folds of the skin. Clinical suspicion can be confirmed by cytological examination, histopathological examination, and direct and indirect immunofluorescence tests. The treatment is performed with systemic corticosteroids, and immunosuppressive drugs may be associated, among them azathioprine and mycophenolate mofetil. More severe cases may benefit from corticosteroids in the form of intravenous pulse therapy, and recent studies have shown a beneficial effect of rituximab, an anti-CD20 immunobiological drug. It is a chronic disease with mortality around 10%, and septicemia is the main cause of death. Patients need long-term and multidisciplinary follow-up.

The impact of COVID-19 infection of patients with autoimmune blistering diseases of the skin

“…Always adjuvant to systemic treatment, topical treatment of lesions aims to reduce pain and prevent secondary infection. It is usually performed with corticosteroid and/or antibiotic creams (12,13). Individual case reports have described a response to topical treatment with the calcineurin inhibitor, tacrolimus.…”

Deep pemphigus (pemphigus vulgaris, pemphigus vegetans and paraneoplastic pemphigus) in dogs, cats and horses: a comprehensive review

“…The female-to-male ratio in Americans with PV is estimated to be 5.0 [ 9 ], which implies that PV is five times more common in American females than males. Other reports estimated a lower female-to-male ratio of 1.5 to 4.0 but the consensus is that PV is more prevalent among women [ 54 ].…”

Dapsone in the Management of Pemphigus and Pemphigoid: Rediscovery of its Long-Lost Efficacy

“…However, since the discovery of its anti-inflammatory properties, it has been used for the treatment of various skin conditions including AMCBD. Pemphigus vulgaris (PV) is the most common blistering disease among the pemphigus group and it accounts for 70% of all pemphigus cases [5,6]. Unlike BP, where mucous membranes are rarely involved [1], the disease process in PV involves both skin and mucous membranes, with the latter being mostly affected first [5].…”

“…The pathophysiology of PV is related to IgG autoantibodies against proteins of the desmosome complex specifically desmoglein (Dsg)1 and/or Dsg3, which hold keratinocytes together. As a result, the intraepithelial junctions are compromised and acantholysis subsequently occurs which is considered a characteristic sign for the pemphigus group of diseases [6]. PV most commonly affects patients between the ages of 40 and 60 years old.…”

“…Skin involvement occurs later, and it is characterized by flaccid fragile blisters that easily rupture, leaving behind painful erosive areas that ooze and bleed easily later forming crusts. The cutaneous involvement can be either localized or generalized and is mostly seen on the trunk, groin, scalp, face, and extremities usually sparing the palms and soles [5,6]. Systemic corticosteroids are the basis of PV treatment with an initial dose of 1-2 mg/kg/day [7].…”