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BMZ and Immune System in normal and AIBD skin

Total questions: 10

Worksheet time: 8mins

Name
Class
Date
1.

What key structural feature of the hemidesmosome interacts with intermediate filaments of cells?

a)

The lamina densa

b)

The inner layer

c)

The outer layer communicating with transmembrane proteins

d)

The anchoring filaments

2.

What are the two domains of type IV collagen that are key to the formation of the collagen network?

a)

The NC1 domain and the 7S domain

b)

The NC1 domain and the triple helical domain

c)

The triple helical domain and the 7S domain

d)

The N-terminal domain and the triple helical domain

3.

What is the specific domain of type XVII collagen (BP180) that IgG autoantibodies target in some forms of bullous pemphigoid?

a)

The C-terminal collagen domain

b)

The NC16A domain

c)

The flexible tail region

d)

The largest collagenous domain 15

4.

The intracellular portion of type XVII collagen binds to which specific component of the hemidesmosome?

a)

Type IV collagen

b)

The α6 integrin

c)

Laminin-332

d)

The β4 integrin

5.

What specific structural feature of type VII collagen monomers is hydroxylated using ascorbic acid as a cofactor for additional stability?

a)

The cysteine residues

b)

The prolines

c)

The glycine residues

d)

The lysine residues

6.

What is the clinical consequence of IgG autoantibodies targeting the NC1 domain of type VII collagen?

a)

Generalized atrophic benign epidermolysis bullosa (GABEB)

b)

Bullous pemphigoid

c)

Cicatricial pemphigoid

d)

Epidermolysis bullosa acquisita

7.

In recessive dystrophic epidermolysis bullosa (DEB), what genetic mechanism often results in the complete absence of anchoring fibrils?

a)

Premature stop codons

b)

Deletions in the NC16A domain

c)

Mutations in the laminin-332 gene

d)

Glycine substitutions in the central repeat region

8.

According to the "desmoglein compensation theory", what determines the clinical phenotype (mucosal vs. mucocutaneous) in pemphigus vulgaris?

a)

The presence of anti-Dsg1 IgG only

b)

The presence of anti-Dsg3 IgG only or in combination with anti-Dsg1 IgG

c)

The ratio of IgG4 to IgG1 antibodies

d)

The involvement of CD4+ T cells

9.

In bullous pemphigoid (BP), T cell and autoantibody reactivities to which specific region of the BP180 protein were related to more extensive disease activity?

a)

The transmembrane domain

b)

The cytoplasmic tail

c)

NH2-terminal epitopes

d)

The collagenous domain

10.

Which immunoglobulin subclass autoantibodies are primarily found in pemphigoid gestationis patients and are noted as complement-fixing?

a)

IgG2 and IgG4

b)

IgG1 and IgG3

c)

IgG1 and IgG4

d)

IgG3 and IgG4