From the Guidelines
Pemphigus vulgaris and bullous pemphigoid are distinct autoimmune blistering skin diseases with important differences in their clinical presentation, pathophysiology, and management. Pemphigus vulgaris affects middle-aged adults (40-60 years) and targets desmoglein proteins in the epidermis, causing fragile, flaccid blisters that rupture easily, leaving painful erosions primarily in the oral mucosa and scalp 1. These blisters form within the epidermis (suprabasal) and demonstrate a positive Nikolsky sign. In contrast, bullous pemphigoid typically affects older adults (>65 years) and targets hemidesmosomes at the dermal-epidermal junction, producing tense, intact blisters on an erythematous or urticarial base, predominantly on the flexural areas and abdomen 1. Mucosal involvement is less common in bullous pemphigoid. Histologically, pemphigus shows intraepidermal acantholysis while bullous pemphigoid shows subepidermal blistering with eosinophilic infiltrates. Immunofluorescence reveals intercellular IgG deposits in pemphigus versus linear deposits along the basement membrane in pemphigoid. Some key differences between the two conditions include:
- Age of onset: Pemphigus vulgaris typically affects middle-aged adults, while bullous pemphigoid affects older adults 1.
- Clinical presentation: Pemphigus vulgaris is characterized by fragile, flaccid blisters that rupture easily, while bullous pemphigoid is characterized by tense, intact blisters 1.
- Histological features: Pemphigus vulgaris shows intraepidermal acantholysis, while bullous pemphigoid shows subepidermal blistering with eosinophilic infiltrates 1.
- Immunofluorescence patterns: Pemphigus vulgaris shows intercellular IgG deposits, while bullous pemphigoid shows linear deposits along the basement membrane 1. Pemphigus vulgaris is generally more severe with higher mortality if untreated, while bullous pemphigoid has a better prognosis but still requires aggressive treatment with corticosteroids, steroid-sparing immunosuppressants, and sometimes rituximab for refractory cases 1. The treatment of pemphigus vulgaris typically involves a combination of oral corticosteroids and immunosuppressive agents, such as azathioprine or mycophenolate mofetil, while the treatment of bullous pemphigoid typically involves superpotent topical corticosteroids and oral corticosteroids, with or without adjuvant immunosuppressive therapy 1. Overall, the management of these conditions requires a comprehensive approach that takes into account the individual patient's needs and circumstances.
From the FDA Drug Label
The FDA drug label does not answer the question.
From the Research
Differences between Pemphigus Vulgaris and Bullous Pemphigoid
- Pemphigus vulgaris is characterized by autoantibodies directed against the cell surface of keratinocytes, producing acantholysis that leads to intraepithelial blisters in the skin and/or mucous membranes 2.
- Bullous pemphigoid, on the other hand, is characterized by autoantibodies present at the dermo-epidermal junction, attacking the hemidesmosomes and causing subepidermal blister formation 2, 3.
- The main subtypes of pemphigus include pemphigus vulgaris, pemphigus foliaceus, and paraneoplastic pemphigus, while bullous pemphigoid is a distinct entity 2, 3.
- Diagnosis of pemphigus vulgaris and bullous pemphigoid is based on clinical manifestations, histological, immunofluorescence, and serological testing, with multivariant enzyme-linked immunosorbent assay systems being developed as practical screening tools 3.
Treatment Differences
- The current first-line treatment of pemphigus vulgaris is based on systemic corticosteroids, often combined with immunosuppressive adjuvants, such as azathioprine, mycophenolate mofetil, and the anti-CD20 monoclonal antibody rituximab 4, 3, 5.
- Treatment of bullous pemphigoid is based on disease extension, with localized and mild forms being treated with superpotent topical corticosteroids or nonimmunosuppressive agents, and systemic corticosteroids being preferred for generalized disease or resistant cases 3.
- Rituximab is a first-line immunomodulatory treatment for moderate to severe pemphigus disease, and its efficacy is higher when administered early in the course of the disease 4, 3, 5.