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Derm > Basement Membrane Zone and Vesiculobullous Diseases > Flashcards

Basement Membrane Zone and Vesiculobullous Diseases Flashcards

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Dermatology Board Prep flashcards
1
Q

What are the 4 parts of the Basement Membrane?

A

1) the plasma membrane and hemidesmosomal plaques of the basal keratinocyte
2) lamina lucida
3) lamina densa
4) sublamina densa

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2
Q

What is BPAG-2?

A

a transmembrane protein made up of XVII collagen

Binds to BPAG1 and integrins (in hemidesmosomes) with laminin 5 located in the laminin 5 located in the lamina densa

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3
Q

What is BPAG-1?

A

an intracytoplasmic protein located within the cytoplasm of the basal keratinocyte hemidesmosomes
binds to keratin intermediate filaments to stabilize the cell

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4
Q

What layer of the basement membrane does the BPAG-2 transverse?

A

lamina lucida

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5
Q

What is the lamina densa composed of?

A

Laminins and collagen IV

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6
Q

What is the key laminin found in the BMZ?

A

laminin 5

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7
Q

Where is Type IV collagen located?

A

lamina densa

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8
Q

What type of collagen is found in the sublamina densa?

A

Type VII

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9
Q

What are immunobullous diseases?

A

inflammation of cells in the epidermis or BMZ

Leads to loss of cell-to-cell adhesion and subsequent bulla formation

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10
Q

What are congenital mechanobullous diseases?

A

There is an absence of structural proteins that prevent normal cell-cell adhesion and promotes blister formation.

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11
Q

What is the Nikolsky sign?

A

Apply gentle pressure to the edges of a blister and pull laterally to determine if a bulla is flaccid or tense

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12
Q

Where is a DIF sample taken from?

A

perilesional site

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13
Q

What is IIF?

A

It tests serum for circulating antibodies

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14
Q

A serological test (DIF) for pemphigus vulgaris is testing against what?

A

IgG antibodies directed against Dsg 1 and 3

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15
Q

A serological test (DIF) for bullous pemphigoid is testing against what?

A

BPAG1 and 2

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16
Q

Describe common features of Pemphigus vulgaris

A

autoimmune blistering disease characterized by flaccid bullae and erosions
oral erosions
crusting of scalp
PAINFUL

17
Q

what will you see histologically in PV?

A

the basal keratinocytes remain connected to the BMZ resulting in a “tombstoning” appearance

18
Q

What is the treatment for PV?

A

systemic steroids and immunosuppressents

19
Q

Describe common features of Bullous Pemphigoid (BP)

A

typically affects adults 60+
itching
tense bullae

20
Q

What does a DIF of BP reveal?

A

a linear staining of the basement membrane with IgG and C3

also seen in CP

21
Q

What is the treatment for BP?

A

high potency topical steroids and oral antibiotics; severe cases may require immunosuppressants

22
Q

Describe common features of Cicatrical Pemphigoid (CP)

A

90% have oral symptoms, 66% have ocular symptoms, and 25 skin (flaccid bullae)
chronic
PAINFUL

23
Q

what is the treatment for CP?

A

potent topical corticosteroids and oral immunosuppressants

24
Q

What are antibodies directed against in CP?

A

BPAG2 and laminin 5

25
Q

What causes inherited mechanobullous diseases?

A

congenital absence of proteins in the BMZ

26
Q

What is Epidermolysis Bullosa (EB)?

A

a group of diseases with bullae and mechanical fragility of the skin

27
Q

What is Epidermyolysis Bullosa simplex?

A

an autosomal dominant disease where epidermal keratins are absent

28
Q

What is junctional Epidermyolysis Bullosa?

A

An autosomal recessive disease with laminin and BPAG2 mutations in the lamina densa

29
Q

What is dystrophic Epidermyolysis Bullosa?

A

an autosomal dominant and recessive disease where collagen VII is absent or abnormal

30
Q

Why does Epidermyolysis Bullosa have a high mortality rate?

A

increased risk of squamous cell carcinoma

31
Q

What disease is associated with celiac disease?

A

Dermatitis Herpeitiformis (DH)

32
Q

What is the treatment for DH?

A

gluten-free diet and dapsone (sulfa-based antibiotic)

33
Q

what are some common features of Dermatitis Herpetiformis (DH)?

A

affects individuals of northern descent
Very itchy
affects the occipital scalp, extensor upper and lower extremities and buttocks
lesions are papules, vesicles, and urticarial plaques

34
Q

What cancer of DH patients at risk of contracting?

A

gastric lymphoma (6%)

Derm (9 decks)

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