6.8.24
Quick Review #145 - #pathology #oralpathology #doctorgallagher #oralsurgery #oralsurgeon #dentist #dentistry #dental
Pemphigus vulgaris (PV) and mucous membrane pemphigoid (MMP) are both autoimmune blistering diseases, but they have distinct differences:
Pemphigus Vulgaris (PV)
1. Pathogenesis:
• PV is caused by autoantibodies against desmogleins (specifically desmoglein 3 and sometimes desmoglein 1), which are components of desmosomes in the epidermis. This leads to acantholysis, where keratinocytes lose adhesion, resulting in intraepidermal blistering.
2. Clinical Presentation:
• PV typically presents with flaccid blisters and erosions on the skin and mucous membranes. The oral mucosa is commonly involved, often being the first site of manifestation.
• The blisters are fragile and often rupture, leading to painful erosions.
3. Histopathology:
• Histologically, PV shows intraepidermal blisters with acantholysis (loss of intercellular connections between keratinocytes).
• Direct immunofluorescence (DIF) shows IgG and C3 deposition in the intercellular spaces of the epidermis.
Mucous Membrane Pemphigoid (MMP)
1. Pathogenesis:
• MMP is caused by autoantibodies against various components of the basement membrane zone (BMZ), such as BP180 (type XVII collagen), BP230, laminin 332, and others. This leads to subepidermal blistering.
2. Clinical Presentation:
• MMP primarily affects mucous membranes, including the oral, ocular, nasopharyngeal, laryngeal, and anogenital regions. Skin involvement is less common.
• The blisters are more resilient compared to PV and lead to scarring, particularly in the eyes, which can result in blindness if not treated.
3. Histopathology:
• Histologically, MMP shows subepidermal blisters with an inflammatory infiltrate.
• DIF shows linear IgG and C3 deposition along the BMZ.
Key Differences
• Level of Blistering: PV causes intraepidermal blisters, while MMP causes subepidermal blisters.
• Target Antigens: PV targets desmogleins in desmosomes, whereas MMP targets components of the BMZ.
• Clinical Manifestations: PV frequently affects both skin and mucous membranes, with mucosal involvement often being the initial presentation. MMP predominantly affects mucous membranes, with significant risk of scarring, especially in the eyes.
• Histopathological Features: PV shows intraepidermal acantholysis with intercellular IgG deposition, while MMP shows subepidermal blistering with linear BMZ deposition.
References:
1. Subadra, K., Sathasivasubramanian, S., & Warrier, S. A. (2021). Oral Pemphigus Vulgaris. Cureus, 13(9), e18005. https://lnkd.in/eUvnZECK
2. Hertl, M. (2001). Autoimmune diseases of the skin: Pathogenesis, diagnosis, management. Springer.
3. Bolognia, J. L., Schaffer, J. V., & Cerroni, L. (2018). Dermatology (4th ed.). Elsevier.
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