Vesiculobullous Disorders (2) Quiz

Which protein is targeted by autoantibodies in Pemphigus Vulgaris?

What is the characteristic feature of Pemphigus Erythematosus compared to other types of pemphigus?

Which disorder is characterized by intraepidermal vesicles associated with acantholysis?

What is the main pathological mechanism in Pemphigus?

Which layer gets separated in a blister according to the text?

Which disease is associated with autoantibodies against Desmoglein 1?

What type of adhesion structures are desmosomes?

Which type of tumor is associated with Paraneoplastic Pemphigus?

In Bullous Pemphigoid, the autoimmune disease is characterized by IgG reacting against which proteins?

Which age group is primarily affected by Bullous Pemphigoid?

Epidermolysis Bullosa involves defects in which network at the dermal/epidermal junction?

Which type of Epidermolysis Bullosa variant is the most common?

Which gene mutation is associated with Dystrophic Epidermolysis Bullosa?

Porphyria results from a disturbance in the metabolism of which substance?

Pemphigus Overview

Autoantibodies in Pemphigus Vulgaris target Desmoglein 3, a protein crucial for cell adhesion in the epidermis.
Pemphigus Erythematosus is characterized by the presence of erythematous lesions, distinguishing it from other pemphigus types which typically feature more extensive blistering.

Mechanisms and Pathology

Intraepidermal vesicles associated with acantholysis are characteristic of Pemphigus disorders.
The main pathological mechanism in Pemphigus involves a breakdown of desmosomal adhesion due to autoantibodies disrupting cell-cell junctions.
In a blister, separation occurs at the suprabasal layer of the epidermis.

Associated Diseases and Conditions

Autoantibodies against Desmoglein 1 are associated with Pemphigus Foliaceus, another form of pemphigus.
Desmosomes are specialized adhesion structures that maintain cell integrity in epithelial tissues.

Paraneoplastic Pemphigus

Paraneoplastic Pemphigus is associated with malignancies, particularly Non-Hodgkin Lymphoma, and can present with severe blisters and mucosal involvement.

Bullous Pemphigoid

In Bullous Pemphigoid, the autoimmune response involves IgG antibodies reacting against proteins in the hemidesmosomes, specifically BP180 and BP230.
This condition primarily affects the elderly, typically those over 60 years of age.

Epidermolysis Bullosa

Epidermolysis Bullosa (EB) involves defects in the anchoring fibrils and type VII collagen at the dermal/epidermal junction.
The most common variant of Epidermolysis Bullosa is Simplex Epidermolysis Bullosa.
Dystrophic Epidermolysis Bullosa is associated with mutations in the type VII collagen gene (COL7A1).

Porphyria

Porphyria results from disturbances in the metabolism of porphyrins, which are essential for hemoglobin synthesis and are sensitive to various environmental factors.

Test your knowledge on vesiculobullous disorders that affect the skin and mucous membranes, including blisters, vesicles, and histologic features for diagnosis. Explore the different causes of blisters and the level of plane of separation in these disorders.