7.12.24

Quick Review #176 - #pathology #oralpathology #doctorgallagher #oralsurgery #oralsurgeon #dentist #dentistry #dental

Pemphigus:

1. Pathogenesis: characterized by autoantibodies against desmogleins, proteins that form the intercellular junctions (desmosomes) between keratinocytes in the epidermis. This leads to a loss of cell-to-cell adhesion, resulting in intraepidermal blisters.

2. Types:

• Pemphigus Vulgaris: The most common type, typically involves mucous membranes and skin. Blisters are flaccid and easily rupture, leaving painful erosions.

• Pemphigus Foliaceus: Primarily affects the skin with superficial blisters, rarely involving mucous membranes.

3. Clinical Features:

• Blisters and erosions on skin and mucous membranes.

• Nikolsky sign positive (skin shears off easily when rubbed).

4. Histopathology:

• Acantholysis (loss of intercellular connections) within the epidermis.

• Intraepidermal blister formation.

5. Diagnosis:

• Direct immunofluorescence showing intercellular deposition of IgG and C3 within the epidermis.

• ELISA for desmoglein antibodies.

Pemphigoid:

1. Pathogenesis: characterized by autoantibodies against components of the basement membrane zone, such as BP180 and BP230, leading to subepidermal blistering.

2. Types:

• Bullous Pemphigoid: The most common type, typically affects elderly individuals and primarily involves the skin.

• Mucous Membrane Pemphigoid: Also known as cicatricial pemphigoid, it predominantly affects mucous membranes and can lead to scarring.

3. Clinical Features:

• Tense, less easily ruptured blisters on skin or mucous membranes.

• Nikolsky sign usually negative.

• Pruritus is common in bullous pemphigoid.

4. Histopathology:

• Subepidermal blister formation.

• Presence of inflammatory infiltrate, often with eosinophils, at the dermal-epidermal junction.

5. Diagnosis:

• Direct immunofluorescence showing linear deposition of IgG and C3 at the basement membrane zone.

• ELISA for BP180 and BP230 antibodies.

References:

1. Bieber, K., Kridin, K., Emtenani, S., Boch, K., Schmidt, E., & Ludwig, R. J. (2021). Milestones in personalized medicine in pemphigus and pemphigoid. Frontiers in Immunology, 11, Article 591971.

2. Bolognia, J. L., Schaffer, J. V., & Cerroni, L. (2018). Dermatology (4th ed.). Elsevier.

3. James, W. D., Elston, D. M., Treat, J. R., & Rosenbach, M. A. (2020). Andrews’ Diseases of the Skin: Clinical Dermatology (13th ed.). Elsevier.

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