Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Derm > autoimmune bullous disease > Flashcards

autoimmune bullous disease Flashcards

You may prefer our related Brainscape-certified flashcards:
Dermatology Board Prep flashcards
0
Q

desmosome is a complex of

A

cadherins (cell adhesion proteins)

linking proteins

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

desmosome

A

cell structured for cell-cell adhesion

anchors intermediate filaments (keratins) to cell membrane

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

Cadherins

A

transmembrane proteins

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

Desmoglein 1 and 3 are restricted to

A

stratified squamous epithelium (spinosum)

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

Dsg1

A

predominant in upper epidermis

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

dsg 3

A

more predominant in lower layers

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

linking proteins

A

cytoplasmic proteins, plakins–attach to intracellular keratin cytoskeletin filaments

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

acantholysis

A

desmosome disruption that reuslts with intraepidermal blisters (flaccid bullae)
–>splitting apart of keratinocytes

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

flaccid bullae

A

bullous lesion that’s fragile and thus usually found without fluid
also seen in herpes (but with multinucleated giant cells)

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

functions of basement membrane zone

A

attaches epidermal basal cell to dermis; acts as permeability barrier; important for tissue repair

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

4 regions of epidermal basement membrane

A

basal keratinocyte
lamina lucida
lamina densa
sublamina densa

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

basal keratinocytes

A

keratins 5,14 (intermediate filaments)

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

lamina lucida

A

composed of anchoring filaments (BP Ag 180)

weakest point of BM zone (cleavage for salt split skin test)

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

anchoring filaments BP Ag 180 in lamina lucida

A

targets for bullous pemphigod

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

lamina densa

A

BM proper seen on H&E
electron dense
Type Iv collagen

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

sublamina densa

A

dermal component of BM zone

contains anchoring fibrils (type VII collagen) and anchoring plaques

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

hemidesmosome

A

dense plaque along basal cell plasma membrane

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

what does the hemidesmosoem complex with

A

lamina lucida

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

what does the hemidesmosome contain

A

important attachment proteins like bullous pemphigoid antigens

19
Q

BP230

A

located in hemidesmosomes

20
Q

BP180

A

collagen type XVII –main BP antigen

also one of several antigens for the disease paraneoplastic pemphigus

21
Q

BP180 location

A

partially in hemidesmosome

partially in lamina lucida

22
Q

pemphigus vulgaris major morbidity

A

fluid loss and secondary bacterial infection due to skin break

23
Q

clinical findings in PV

A

usually present as mucosal erosions in 100% and upper body skin in 50%
flaccid bullae

24
Q

Nikolsky sign

A

upper layers of skin near bullous easily slip by slight pressure or rubbing leaving glistening skin

25
Q

asboe hansen sign

A

pressure on intact bulla extends bulla

26
Q

rupture of PV

A

form erosions

27
Q

healing of PV lesions

A

hyperpigmentation but NOT scarring because they are ABOVE LAMINCA LUCIDA

28
Q

diseases that reach lamina densa

A

leave a scar

29
Q

pathogenesis of PV

A

intercellular abs against desmoglein 1 and desmoglein 3 (members of cadherin family)

30
Q

histology of PV

A

tombstoning, intraepidermal split, acantholysis

31
Q

dx techniques for PV

A 
skin bx of skin next to bulla
skin bx for IF 
skin bx then salt split skin IF
serum for indirect IF (positive in 80-90% in early fz or remission)
electron microscopy
32
Q

gold standard of PV diagnositix

A

perilesional (special fixative required)
-intercellular IgG (chicken wire)
C3 deposition in acnahtolytic areas

33
Q

Tx of PV

A

prednisone
rituximab
immunsupps

34
Q

pemphigus foliaceus

A

superificial variant of pemphigus of the upper chest and scalp distribution
more benign course and oral lesions rare

35
Q

pathophys of pemiphigus foliaceous

A

ant-dsg 1 autoantibodies

endemic via fly in brazil

36
Q

paraneoplastic pemphigus

A 
very rare
associated with thymoma or NHL or CLL
mucosal invovlment is classic, but bullae on skin and erythema multiforme
*usually both ocular and lip involvment
IgA
37
Q

bullous pemphigoid demographic

A

dz of elderly (65-75) >80% are over 60

38
Q

clinical picture of bullous pemphigoid

A

large, tense bullae with much less erosions on groin axillae thighs and FLEXOR forearms
neg nikolsy and asboe hansen
itchy***
oral involvment much lower

39
Q

patho of bullous pemphigoid

A

blister patho is deeper (within lamina lucida)–>no acantholysis
subepidermal blister with eosinopgils

40
Q

antigens of BP

A

proteins of hemides BPA1 ,BPAg2 (main)

41
Q

dx BP

A

direct IF for linear c3 and Igg in lamina lucida

IIF -serum titers do not correspond to dz activity

42
Q

tx of BP

A

potent topical steroids for limited disease
prednisone
rituximab
immunosups

43
Q

how long till BP clers

A

5-6 years

44
Q

epidermolysis bullosa

A

NOT autoimmune–heterogeneous, rare, inherited disorder

45
Q

classification of EB by

A

where blisters arise
genetic mode of transmission
clinical pheno

46
Q

forms of EB

A

mechanical fragility
tense, clear fluid filled blisters
erosions and crusts
scarring

Derm (15 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions