autoimmune bullous disease

Question 0

desmosome is a complex of

Answer 0

cadherins (cell adhesion proteins)

linking proteins

Question 1

desmosome

Answer 1

cell structured for cell-cell adhesion

anchors intermediate filaments (keratins) to cell membrane

Question 2

Cadherins

Answer 2

transmembrane proteins

Question 3

Desmoglein 1 and 3 are restricted to

Answer 3

stratified squamous epithelium (spinosum)

Question 4

Dsg1

Answer 4

predominant in upper epidermis

Question 5

dsg 3

Answer 5

more predominant in lower layers

Question 6

linking proteins

Answer 6

cytoplasmic proteins, plakins–attach to intracellular keratin cytoskeletin filaments

Question 7

acantholysis

Answer 7

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

Question 8

flaccid bullae

Answer 8

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

Question 9

functions of basement membrane zone

Answer 9

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

Question 10

4 regions of epidermal basement membrane

Answer 10

basal keratinocyte
lamina lucida
lamina densa
sublamina densa

Question 11

basal keratinocytes

Answer 11

keratins 5,14 (intermediate filaments)

Question 12

lamina lucida

Answer 12

composed of anchoring filaments (BP Ag 180)

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

Question 13

anchoring filaments BP Ag 180 in lamina lucida

Answer 13

targets for bullous pemphigod

Question 14

lamina densa

Answer 14

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

Question 15

sublamina densa

Answer 15

dermal component of BM zone

contains anchoring fibrils (type VII collagen) and anchoring plaques

Question 16

hemidesmosome

Answer 16

dense plaque along basal cell plasma membrane

Question 17

what does the hemidesmosoem complex with

Answer 17

lamina lucida

Question 18

what does the hemidesmosome contain

Answer 18

important attachment proteins like bullous pemphigoid antigens

Question 19

BP230

Answer 19

located in hemidesmosomes

Question 20

BP180

Answer 20

collagen type XVII –main BP antigen

also one of several antigens for the disease paraneoplastic pemphigus

Question 21

BP180 location

Answer 21

partially in hemidesmosome

partially in lamina lucida

Question 22

pemphigus vulgaris major morbidity

Answer 22

fluid loss and secondary bacterial infection due to skin break

Question 23

clinical findings in PV

Answer 23

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

Question 24

Nikolsky sign

Answer 24

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

Question 25

asboe hansen sign

Answer 25

pressure on intact bulla extends bulla

Question 26

rupture of PV

Answer 26

form erosions

Question 27

healing of PV lesions

Answer 27

hyperpigmentation but NOT scarring because they are ABOVE LAMINCA LUCIDA

Question 28

diseases that reach lamina densa

Answer 28

leave a scar

Question 29

pathogenesis of PV

Answer 29

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

Question 30

histology of PV

Answer 30

tombstoning, intraepidermal split, acantholysis

Question 31

dx techniques for PV

Answer 31

``` 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 ```

Question 32

gold standard of PV diagnositix

Answer 32

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

Question 33

Tx of PV

Answer 33

prednisone rituximab immunsupps

Question 34

pemphigus foliaceus

Answer 34

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

Question 35

pathophys of pemiphigus foliaceous

Answer 35

ant-dsg 1 autoantibodies | endemic via fly in brazil

Question 36

paraneoplastic pemphigus

Answer 36

``` 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 ```

Question 37

bullous pemphigoid demographic

Answer 37

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

Question 38

clinical picture of bullous pemphigoid

Answer 38

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

Question 39

patho of bullous pemphigoid

Answer 39

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

Question 40

antigens of BP

Answer 40

proteins of hemides BPA1 ,BPAg2 (main)

Question 41

dx BP

Answer 41

direct IF for linear c3 and Igg in lamina lucida | IIF -serum titers do not correspond to dz activity

Question 42

tx of BP

Answer 42

potent topical steroids for limited disease prednisone rituximab immunosups

Question 43

how long till BP clers

Answer 43

5-6 years

Question 44

epidermolysis bullosa

Answer 44

NOT autoimmune--heterogeneous, rare, inherited disorder

Question 45

classification of EB by

Answer 45

where blisters arise genetic mode of transmission clinical pheno

Question 46

forms of EB

Answer 46

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