Epidermal Proteins

Question 1

K1/K10

Answer 1

Suprabasal keratinocytes.

EHK (bullous CIE)
Unna Thost PPK (non-epidermolytic PPK)

Question 2

K1/K9

Answer 2

Palmoplantar suprabasal keratinocytes

Vorner PPK (epidermolytic PPK)

Question 3

K2/K10

Answer 3

Granular and upper spinous layer

Icthyosa Bullosa of Siemens

Question 4

K3/K12

Answer 4

Cornea

Meesman corneal dystrophy

Question 5

K4/K13

Answer 5

Mucosal epithelium

White sponge nevus

Question 6

K5/K14

Answer 6

Basal keratinocytes

Dowling-Degos disease
EBS

Question 7

K6a/K16

Answer 7

Outer root sheath

Pachyonychia Congenita Type 1
Hyperproliferative conditions (psoriasis)

Question 8

K6b/K17

Answer 8

Nail bed

Pachyonychia Congenita Type 2

Question 9

K8/K18

Answer 9

Simple epithelium

Cryptogenic cirrhosis

Question 10

K81/K86

Answer 10

Hair

Monilethrix

Question 11

K13

Answer 11

Warts in transplant recipients

Question 12

K19

Answer 12

Stem cells

Question 13

Plakoglobin

Answer 13

Desmosome

Naxos syndrome

Question 14

Desmocolin

Answer 14

Desmosome

Subcorneal pustular dermatosis

Question 15

Desmoplakin

Answer 15

Desmosome

Carvajal syndrome

Question 16

Desmoglein 1

Answer 16

Desmosome

Pemphigus Folliaceous
Striate PPK

Question 17

Desmoglein 3

Answer 17

Desmosome

Pemphigus Vulgaris

Question 18

Plakophilin

Answer 18

Desmosome

Ectodermal dysplasia with skin fragility

Question 19

B-catenin

Answer 19

Adherens junctions

Pilomatricomas
Myotonic dystrophy

Question 20

GJB2

Answer 20

Connexin 26
Gap Junction

KID Syndrome
Vohwinkel’s PPK

Question 21

GJB6

Answer 21

Connexin 30
Gap Junction

Hidrotic ectodermal dysplasia (Clouston)

Question 22

GJB3

GJB4

Answer 22

Connexin 30.3
Connexin 31
Gap Junction

Erythrokeratododerma Variabilis (Mendes de Costa)

Question 23

a6B4 integrin

Answer 23

Hemidesmosome

Junctional EB with pyloric atresia
Cicatricial pemphigoid (ocular)

Question 24

Plectin

Answer 24

Hemidesmosome

EB Simplex with muscular dystrophy

Question 25

BPAG1 | BPAG2

Answer 25

Hemidesmosome Bullous pemphigoid

Question 26

Futcher's lines

Answer 26

lines of pigmentary demarcation (ventral and dorsal surfaces, like front and back of hand)

Question 27

Lipid and calcium changes moving up the epidermis

Answer 27

``` increasing calcium (more desmosomes) increasing sphingolipids (important for differentiation) decreasing phospholipids ```

Question 28

Time for epidermal turnover | Time for stratum corneum turnover

Answer 28

45-60 days | 2 weeks

Question 29

Type I and Type II keratins

Answer 29

Type 1 = acidic epithelial K9-K28 hair K31-K40 Type 2 = basic epithelial K1-K8, K71-K80 hair K81-K86

Question 30

Lamellar Granules

Answer 30

Stratum spinosum Phospholipids, cholesterol sulfate, glucosylceramides Proteases Human beta defensin 2 Decreased, harlequin icthyosis No steroid sulfatase, x-linked icthyosis Increased (but abnormal), CIE

Question 31

Desmosome Junctions

Answer 31

Ca-depending, scaffolding, stress bearing Desmosomal Cadherins (transmembrane) Desmoglein 1/3 Desmocolin 1/2 Plaque proteins Plakoglobin (gamma catenin) - also in adherens junctions Desmoplakin 1/2 Envoplakin

Question 32

Adherens Junctions

Answer 32

Adhesion Transmembrane, classical cadherins (E and P) alpha, beta, and gamma cadherin