Overview
Pemphigus foliaceus is an autoimmune blistering disorder characterized by superficial acantholytic blistering, primarily affecting the skin without significant mucosal involvement 3.Diagnosis
Clinical Presentation: Superficial blisters and erosions, often with a tendency to rupture easily 3.
Direct Immunofluorescence: Demonstrates intercellular IgG deposition in the epidermis 3.
Indirect Immunofluorescence: Confirms circulating autoantibodies against desmoglein 1 3.
Biopsy: Histopathology shows acantholysis without significant inflammatory cell infiltration 3.Management
First-Line Treatment: High-dose corticosteroids (e.g., prednisone 1 mg/kg/day) 3.
Adjunctive Therapy:
- Immunosuppressants: Azathioprine or mycophenolate mofetil to reduce steroid dose and maintain remission 3.
- Antimalarials: Hydroxychloroquine may be considered for adjunctive benefit in some cases 3.Special Populations
Elderly: Higher vigilance required due to potential for more severe presentations and complications 3.
Comorbidities: Management should consider interactions and impact on existing conditions, particularly when using immunosuppressants 3.Key Recommendations
Use direct immunofluorescence and indirect immunofluorescence for definitive diagnosis (Evidence: Moderate 3).
Initiate treatment with high-dose corticosteroids as first-line therapy (Evidence: Moderate 3).
Consider adjunctive immunosuppressive therapy to manage refractory cases or reduce corticosteroid dependency (Evidence: Moderate 3).
Include pemphigus foliaceus in the differential diagnosis of bullous erythroderma, especially in severe presentations (Evidence: Weak 3).References
1 Martins Mda C, Carneiro MG, Utzig JB, Kaled Neta EL, Pachnicki MA, Silva de Castro CC. Scientific output of Brazilian dermatologists during the last 25 years in the five highest impact factor journals in dermatology. Anais brasileiros de dermatologia 2012. link
2 Steffen C, Thomas D. The men behind the eponym: Francis E. Senear, Barney Usher, and the Senear-Usher syndrome. The American Journal of dermatopathology 2003. link
3 Nousari HC, Moresi M, Klapper M, Anhalt GJ. Nonendemic pemphigus foliaceus presenting as fatal bullous exfoliative erythroderma. Cutis 2001. link