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Dermatology > Bullous Disease > Flashcards

Bullous Disease Flashcards

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Dermatology Board Prep flashcards
1
Q

Striate palmoplantar keratoderma type 1

A

mutation in dsg 1

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2
Q

Armadillo proteins

A

plaque proteins
plakoglobin, plakophillin
Beta catenin

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3
Q

Ectodermal dysplasia with skin fragility

A

plakophillin1

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4
Q

Plakoglobin

A

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

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5
Q

Cadherins

A

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

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6
Q

Desmoglein 1

A

160kDa

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7
Q

desmocollin

A

Antigen in IgA pemphigus

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8
Q

Dsg1

A

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

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9
Q

Desmoglein 3

A

130kDa

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10
Q

Transglutaminase1

A

Lamellar ichthyosis

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11
Q

Transglutaminase3

A

DH

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12
Q

Junctional EB with pyloric atrestia

A

alpha-6 Beta-4 mutation

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13
Q

Beta4 integrin

A

Ocular cicatricial pemphigoid Antigen

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14
Q

Type 1 intermediate filaments

A

Acidic K9-20

Chrome 17

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15
Q

Type 2 intermediate filaments

A

Basic 1-8

Chrome 12

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16
Q

Type 3 intermediate filaments

A

Vimentin
glial fibrillary acidic protein
Desmin
Peripherin

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17
Q

Type 4 intermediate filaments

A

Neurofilaments

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18
Q

Type 5 intermediate filaments

A

Nuclear laminins

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19
Q

Type 6 intermediate filaments

A

nestin

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20
Q

Lamin a

A

progeria

21
Q

Keratin 9

A

Epidermolytic PPK

22
Q

Keratin 1 and 16

A

Non epidermolytic PPK

23
Q

Keratin 4/13

A

White sponge nevus of Cannon

24
Q

Keratin 3/12

A

Corneal dystrophy

25
Q

Keratin 1

A

Ichthyosis hystrix

26
Q

Plakins

A 
BPAg1 (230kDa)
Plectin (500kDa)
Desmoplakin 1 (250)
Desmoplakin 2 (210)
Envoplakin (210)
Periplakin (190)
27
Q

EBS with muscular dystrophy

A

plectin

28
Q

Paraneoplastic pemphigus

A

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

29
Q

Stevens Johnson Syndrome

A

Desmoplakin 1 and 2

30
Q

Bullous Pemphigoid

A

BPAg 2 NC16A domain

BPAg1

31
Q

Adherens Junction

A 
Quick but weak adhesion, important for cell signaling
E and P cadherin
alpha and beta catenin
Vinculin and radixin
Plakoglobin
32
Q

Desmosomes

A

Slow but strong

33
Q

Laminin 5

A

400-440 kDa
JEB, herlitz
Cicatricial pemphigoid–antigen

34
Q

Connexin 26

A

GJB

Vohwinkls, PPK with deaf, KID

35
Q

Connexin 30.3 and 31

A

EKV

36
Q

Connexin 30

A

Clouston Syndrome–hidrotic ED

37
Q

BPAg2

A

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

38
Q

BPAg2-C terminal domain

A

Cicatricial pemphigoid, mucosal and skin

39
Q

BPAg2-NC16A

A

BP and Herpes Gestastionis

40
Q

Lamina densa

A

Nidogen (entactin)

Type IV collagen and heparin sulfate

41
Q

Lamina lucida

A

Laminin 5 and anchoring filaments

42
Q

Granular layer

A

Tight junctions

43
Q

Malignancy associated Cicatricial Pemphigoid

A

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

44
Q

IgA pemphigus (subcorneal pustular type)

A

serpiginous pustules

Against desmocollins

45
Q

IgA pemphigus (intraepidermal neutrophilic type)

A

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

46
Q

Pemphigus vegetans Neumann type

A

starts and ends like PV then turn into vegetating malodorous plaques

47
Q

Pemphigus vegetans Hallopeau type

A

start with pustules then then turn into vegetating malodorous plaques

48
Q

Ritters disease

A

Staph group 2, phage 71

Dermatology (20 decks)

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