2. Vesiculobullous Disorders Flashcards

1
Q

What are vesiculobullous disorders?

A

Heterogeneous group of disorders affecting the skin or mucous membranes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Vesiculobullous disorders may come as?

A

Blister
Vesicles

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Blisters are?

A

Fluid-filled cavity within or beneath the epidermis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Vesicles are?

A

< 1 cm

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Bullas are?

A

> 1 cm

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What can blisters be secondary to?

A

Herpes infection
spongiotic dermatitis
burns
lupus erythematous
lichen planus
scleroderma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What are intercellular bridges?

A

a key morphological feature of keratinocytes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What are desmosomes?

A

a strong intercellular adhesion structure

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What are hemidesmosome?

A

a strong adhesion structure between keratinocytes and basement membrane

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What are the types of inflammatory blistering disorders?

A

Pemphigus
pemphigus vulgaris
Paraneoplastic pemphigus
Bullous pemphigoid

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What are the types of non-inflammatory blistering disorders?

A

Epidermolysis Bullosa
Epidermolysis Bullosa Simplex
Epidermolysis Bullosa Dystrophic Type
Porphyria
Herpes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What are the 5 types of Pemphigus?

A

Vulgaris
Erythematosus
Foliaceus
Vegetans
Paraneoplastic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Pemphigus may be associated with?

A

internal malignancies, including thymoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Intraepidermal vesicles are associated with?

A

acantholysis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What is the pathogenesis of Pemphigus?

A

Autoantibody, IgG, against desmoglein 1 and 3

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

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

A

True

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What areas are involved in pemphigus vulgaris?

A

BOTH mucosa and skin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Describe the fluid of pemphigus vulgaris

A

clear

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What is usually accompanied with pemphigus vulgaris’ healing?

A

post-inflammatory hyperpigmentation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

During pemphigus vulgaris, what may cause death?

A

staphylococcal infection
pulmonary embolism

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Describe the pathological features of pemphigus vulgaris

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
Q

Pemphigus erythematosus appears where?

A

localized areas, resemble lupus malar rash on face, and may be considered as a localized less severe form of Pemphigus foliaceus

26
Q

Paraneoplastic pemphigus is associated with what internal tumors?

A

lymphoma
thymoma
carcinoma of the bronchus, pancreas, and breast

27
Q

Paraneoplastic pemphigus involves what areas

A

BOTH oral mucosa and skin

28
Q

Describe bullous pemphigoid

A

Subepidermal, nonacantholytic, autoimmune disease

29
Q

What antibodies are present with bullous pemphigoid?

A

IgG

30
Q

IgG antibodies are with what hemidesmosoal proteins such as

A

BP230 and BP180

31
Q

bullous pemphigoid is mostly seen in what population?

A

elderly

32
Q

(T?F) Bullous pemphigoid involves the oral or ocular mucosa?

A

False…rarely

33
Q

Does bullous pemphigoid rupture easily?

A

No

34
Q

How does bullous pemphigoid heal?

A

without scarring unless infected

35
Q

Epidermolysis Bullosa is?

A

a group of inherited disorders

36
Q

When does Epidermolysis Bullosa primarily occur?

A

onset at birth or early infancy

37
Q

How is eidermolysis Bullosa classified?

A

based on level of tissue separation

38
Q

What form of Epidermolysis Bullosa is most common

A

simplex

39
Q

Is Epidermolysis Bullosa simplex is autosomal dominant?

A

yes

40
Q

Where are the mutations located in Epidermolysis Bullosa simplex?

A

keratin 14 or 5

41
Q

What is the result of the degeneration of the basal layer in Epidermolysis Bullosa?

A

clinical bullae

42
Q

What time of separation is present in Epidermolysis Bullosa simplex?

A

intraepidermal

43
Q

What are the pathological findings of Epidermolysis Bullosa Simplex ?

A

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
Q

blisters are located where in Epidermolysis Bullosa Junctional Type?

A

lamina lucida

45
Q

In Epidermolysis Bullosa Junctional Type, how may the skin appear?

A

normal

46
Q

where is the defect located in Epidermolysis Bullosa Junctional Type?

A

laminin 5

47
Q

Epidermolysis Bullosa Junctional Type is autsomal ______?

A

recessive

48
Q

Epidermolysis Bullosa Dystrophic Type may be autosomal ____?

A

dominant or recessive

49
Q

Where does Epidermolysis Bullosa Dystrophic Type blister?

A

beneath the lamina densa

50
Q

Epidermolysis Bullosa Dystrophic Type has what pathological features?

A

o Few amount of anchoring fibrils beneath the lamina densa

51
Q

What is Porphyria?

A

Non-inflammatory blistering disorder due to disturbance of porphyrin metabolism

52
Q

What is the hereditary pattern of porphyria?

A

Autosomal dominant except congenital erythropoietic porphyria (autosomal recessive)

53
Q

What is a clinical feature of porphyria?

A

o Delta-aminolefulinic acid (DALA): Pain

54
Q

What is herpes?

A

Painful diseases caused by herpes simplex virus (HSV) or varicella zoster virus (VZV, also causes chickenpox)

55
Q

What are the pathological features of herpes?

A

Grouped vesicles

Multinucleated cells with distinct nuclear inclusions