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.
Plastic surgery procedures in which large detachment is planned in patients with a moderate risk of deep venous thrombosis should be evaluated with regard to the risk and benefit of thromboembolism prophylaxis. Other measures must be applied and eventually contraindicate a surgical procedure. Further research is needed to complement the data from this work.
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