Bullous Disease

Question 1

Striate palmoplantar keratoderma type 1

Answer 1

mutation in dsg 1

Question 2

Armadillo proteins

Answer 2

plaque proteins
plakoglobin, plakophillin
Beta catenin

Question 3

Ectodermal dysplasia with skin fragility

Answer 3

plakophillin1

Question 4

Plakoglobin

Answer 4

85kDa
Naxos-mutation
Precips with dsgs in P foliaceous and vulgaris

Question 5

Cadherins

Answer 5

Calcium dependent adhesion molecules
E,P,N are classic
Dsg1,3, and desmocollin

Question 6

Desmoglein 1

Answer 6

160kDa

Question 7

desmocollin

Answer 7

Antigen in IgA pemphigus

Question 8

Dsg1

Answer 8

Antigen in P foliaceous and Ritter’s disease (SSSS)

Question 9

Desmoglein 3

Answer 9

130kDa

Question 10

Transglutaminase1

Answer 10

Lamellar ichthyosis

Question 11

Transglutaminase3

Answer 11

DH

Question 12

Junctional EB with pyloric atrestia

Answer 12

alpha-6 Beta-4 mutation

Question 13

Beta4 integrin

Answer 13

Ocular cicatricial pemphigoid Antigen

Question 14

Type 1 intermediate filaments

Answer 14

Acidic K9-20

Chrome 17

Question 15

Type 2 intermediate filaments

Answer 15

Basic 1-8

Chrome 12

Question 16

Type 3 intermediate filaments

Answer 16

Vimentin
glial fibrillary acidic protein
Desmin
Peripherin

Question 17

Type 4 intermediate filaments

Answer 17

Neurofilaments

Question 18

Type 5 intermediate filaments

Answer 18

Nuclear laminins

Question 19

Type 6 intermediate filaments

Answer 19

nestin

Question 20

Lamin a

Answer 20

progeria

Question 21

Keratin 9

Answer 21

Epidermolytic PPK

Question 22

Keratin 1 and 16

Answer 22

Non epidermolytic PPK

Question 23

Keratin 4/13

Answer 23

White sponge nevus of Cannon

Question 24

Keratin 3/12

Answer 24

Corneal dystrophy

Question 25

Keratin 1

Answer 25

Ichthyosis hystrix

Question 26

Plakins

Answer 26

``` BPAg1 (230kDa) Plectin (500kDa) Desmoplakin 1 (250) Desmoplakin 2 (210) Envoplakin (210) Periplakin (190) ```

Question 27

EBS with muscular dystrophy

Answer 27

plectin

Question 28

Paraneoplastic pemphigus

Answer 28

Desmo/envo/peri plakin and BPAg1 Plectin, 170kDa ag, Desmoglein1 and 2 Histo like EM and PV #1 NHL

Question 29

Stevens Johnson Syndrome

Answer 29

Desmoplakin 1 and 2

Question 30

Bullous Pemphigoid

Answer 30

BPAg 2 NC16A domain | BPAg1

Question 31

Adherens Junction

Answer 31

``` Quick but weak adhesion, important for cell signaling E and P cadherin alpha and beta catenin Vinculin and radixin Plakoglobin ```

Question 32

Desmosomes

Answer 32

Slow but strong

Question 33

Laminin 5

Answer 33

400-440 kDa JEB, herlitz Cicatricial pemphigoid--antigen

Question 34

Connexin 26

Answer 34

GJB | Vohwinkls, PPK with deaf, KID

Question 35

Connexin 30.3 and 31

Answer 35

EKV

Question 36

Connexin 30

Answer 36

Clouston Syndrome--hidrotic ED

Question 37

BPAg2

Answer 37

Generalized atrophic benign EB--mutation Lichen planus pemphigoids--Antigen LInear IgA disease--part is antigen

Question 38

BPAg2-C terminal domain

Answer 38

Cicatricial pemphigoid, mucosal and skin

Question 39

BPAg2-NC16A

Answer 39

BP and Herpes Gestastionis

Question 40

Lamina densa

Answer 40

Nidogen (entactin) | Type IV collagen and heparin sulfate

Question 41

Lamina lucida

Answer 41

Laminin 5 and anchoring filaments

Question 42

Granular layer

Answer 42

Tight junctions

Question 43

Malignancy associated Cicatricial Pemphigoid

Answer 43

Laminin 5 (=epiligrin, BM600, kalinin, nicein)

Question 44

IgA pemphigus (subcorneal pustular type)

Answer 44

serpiginous pustules | Against desmocollins

Question 45

IgA pemphigus (intraepidermal neutrophilic type)

Answer 45

flacid bullae that enlarge peripherally to sunflower, intertriginous areas, pruritus common

Question 46

Pemphigus vegetans Neumann type

Answer 46

starts and ends like PV then turn into vegetating malodorous plaques

Question 47

Pemphigus vegetans Hallopeau type

Answer 47

start with pustules then then turn into vegetating malodorous plaques

Question 48

Ritters disease

Answer 48

Staph group 2, phage 71