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Published on: December 19, 2014
Pemphigus vulgaris
Adriana Maria Porro1, Camila Arai Seque1, Maria Carolina Corsi Ferreira1
1Department of Dermatology, Escola Paulista de Medicina, Universidade Federal de São Paulo, São Paulo, SP, Brazil.
Pemphigus vulgaris is a severe autoimmune skin disease caused by antibodies against desmogleins. Treatment involves corticosteroids and immunosuppressants, with rituximab showing promise for severe cases.
Area of Science:
- Dermatology
- Autoimmunology
- Immunodermatology
Background:
- Pemphigus vulgaris is a chronic autoimmune bullous dermatosis.
- It is characterized by autoantibodies against desmogleins 1 and 3.
- This is the most frequent and severe form of pemphigus, affecting individuals typically between 40 and 60 years of age.
Purpose of the Study:
- To provide a comprehensive overview of Pemphigus Vulgaris.
- To discuss its clinical presentation, diagnosis, and management.
- To highlight the chronic nature and mortality associated with the disease.
Main Methods:
- Clinical suspicion is confirmed through cytological examination.
- Histopathological examination aids in diagnosis.
- Direct and indirect immunofluorescence tests are crucial for confirmation.
Main Results:
- The disease typically begins with oral mucosal blisters and erosions.
- Skin lesions manifest as flaccid blisters, which can be widespread.
- A variant, pemphigus vegetans, presents with vegetating lesions in skin folds.
Conclusions:
- Treatment includes systemic corticosteroids and immunosuppressive drugs like azathioprine and mycophenolate mofetil.
- Intravenous pulse corticosteroid therapy and rituximab are options for severe cases.
- Pemphigus vulgaris is a chronic condition with a 10% mortality rate, primarily due to septicemia, necessitating long-term multidisciplinary care.
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