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2. Vesiculobullous Disorders Flashcards

1
Q

What are vesiculobullous disorders?

A

Heterogeneous group of disorders affecting the skin or mucous membranes

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2
Q

Vesiculobullous disorders may come as?

A

Blister
Vesicles

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3
Q

Blisters are?

A

Fluid-filled cavity within or beneath the epidermis

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4
Q

Vesicles are?

A

< 1 cm

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5
Q

Bullas are?

A

> 1 cm

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6
Q

What can blisters be secondary to?

A

Herpes infection
spongiotic dermatitis
burns
lupus erythematous
lichen planus
scleroderma

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7
Q

What are some key features for the diagnosis of vesiculobullous disorders?

A

*Level of plane of separation
*Presence or absence of acantholysis
*Characterization of inflammatory infiltrate
*Immunofluorescence pattern
*Intercellular bridges, a key morphological feature of keratinocytes

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8
Q

What are intercellular bridges?

A

a key morphological feature of keratinocytes

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9
Q

What are desmosomes?

A

a strong intercellular adhesion structure

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10
Q

What are hemidesmosome?

A

a strong adhesion structure between keratinocytes and basement membrane

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11
Q

What are the types of inflammatory blistering disorders?

A

Pemphigus
pemphigus vulgaris
Paraneoplastic pemphigus
Bullous pemphigoid

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12
Q

What are the types of non-inflammatory blistering disorders?

A

Epidermolysis Bullosa
Epidermolysis Bullosa Simplex
Epidermolysis Bullosa Dystrophic Type
Porphyria
Herpes

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13
Q

What are the 5 types of Pemphigus?

A

Vulgaris
Erythematosus
Foliaceus
Vegetans
Paraneoplastic

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14
Q

Pemphigus may be associated with?

A

internal malignancies, including thymoma

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15
Q

Intraepidermal vesicles are associated with?

A

acantholysis

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16
Q

What is the pathogenesis of Pemphigus?

A

Autoantibody, IgG, against desmoglein 1 and 3

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17
Q

(T/F) Pemphigus vulgaris is the most common type?

A

True

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18
Q

What areas are involved in pemphigus vulgaris?

A

BOTH mucosa and skin

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19
Q

Describe the fluid of pemphigus vulgaris

A

clear

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20
Q

What is usually accompanied with pemphigus vulgaris’ healing?

A

post-inflammatory hyperpigmentation

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21
Q

During pemphigus vulgaris, what may cause death?

A

staphylococcal infection
pulmonary embolism

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22
Q

Describe the pathological features of pemphigus vulgaris

A

Suprabasilar bullae with single row of basal cells resides on basement membrane

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23
Q

What are the clinical features of pemphigus vulgaris?

A

skin blisters filled with clear fluid
ruptured oral mucosa bullae appearing as ulcer

24
Q

Pemphigus erythematosus is referred to ask what syndrome?

A

Senear-Usher

25
Pemphigus erythematosus appears where?
localized areas, resemble lupus malar rash on face, and may be considered as a localized less severe form of Pemphigus foliaceus
26
Paraneoplastic pemphigus is associated with what internal tumors?
lymphoma thymoma carcinoma of the bronchus, pancreas, and breast
27
Paraneoplastic pemphigus involves what areas
BOTH oral mucosa and skin
28
Describe bullous pemphigoid
Subepidermal, nonacantholytic, autoimmune disease
29
What antibodies are present with bullous pemphigoid?
IgG
30
IgG antibodies are with what hemidesmosoal proteins such as
BP230 and BP180
31
bullous pemphigoid is mostly seen in what population?
elderly
32
(T?F) Bullous pemphigoid involves the oral or ocular mucosa?
False...rarely
33
Does bullous pemphigoid rupture easily?
No
34
How does bullous pemphigoid heal?
without scarring unless infected
35
Epidermolysis Bullosa is?
a group of inherited disorders
36
When does Epidermolysis Bullosa primarily occur?
onset at birth or early infancy
37
How is eidermolysis Bullosa classified?
based on level of tissue separation
38
What form of Epidermolysis Bullosa is most common
simplex
39
Is Epidermolysis Bullosa simplex is autosomal dominant?
yes
40
Where are the mutations located in Epidermolysis Bullosa simplex?
keratin 14 or 5
41
What is the result of the degeneration of the basal layer in Epidermolysis Bullosa?
clinical bullae
42
What time of separation is present in Epidermolysis Bullosa simplex?
intraepidermal
43
What are the pathological findings of Epidermolysis Bullosa Simplex ?
Intact stratum corneum and upper epidermis, with vesicle formation in the lower epidermis at the basal layer caused by degeneration of individual epidermal cells
44
blisters are located where in Epidermolysis Bullosa Junctional Type?
lamina lucida
45
In Epidermolysis Bullosa Junctional Type, how may the skin appear?
normal
46
where is the defect located in Epidermolysis Bullosa Junctional Type?
laminin 5
47
Epidermolysis Bullosa Junctional Type is autsomal ______?
recessive
48
Epidermolysis Bullosa Dystrophic Type may be autosomal ____?
dominant or recessive
49
Where does Epidermolysis Bullosa Dystrophic Type blister?
beneath the lamina densa
50
Epidermolysis Bullosa Dystrophic Type has what pathological features?
o Few amount of anchoring fibrils beneath the lamina densa
51
What is Porphyria?
Non-inflammatory blistering disorder due to disturbance of porphyrin metabolism
52
What is the hereditary pattern of porphyria?
Autosomal dominant except congenital erythropoietic porphyria (autosomal recessive)
53
What is a clinical feature of porphyria?
o Delta-aminolefulinic acid (DALA): Pain
54
What is herpes?
Painful diseases caused by herpes simplex virus (HSV) or varicella zoster virus (VZV, also causes chickenpox)
55
What are the pathological features of herpes?
Grouped vesicles Multinucleated cells with distinct nuclear inclusions

Integument (6 decks)

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