Fitz9. Ch14: Skin Barrier

Question 1

Which layer of the stratum granulosum does the right junctions seal the intercellular spaces

Answer 1

SG2

Question 2

Cornified envelope is composed of 3 things

Answer 2

Envoplakin
Periplakin
Involucrin

These are expressed in the granular layer

Question 3

Defect in transglutamidase 1 and loricrin; acylceramide

Answer 3

Congenital ichthyosis

Question 4

ABCA12 functions in cellular lipid trafficking on lamellar bodies.
Defect in ABCA12 Is seen in.

Answer 4

Harlequin ichthyosis

Lamellar bodies are stored in stratum granulosum 3

Question 5

Deficiency in NSDHL

Answer 5

CHILD syndrome

Question 6

defects of SPINK5 (LEKTI) due to enhanced degradation of corneodesmosomes by kallikreins

Answer 6

Netherton syndrome

Corneodesmosin is secreted by lamellar bodies surrounding stratum granulosum 1

Question 7

Defect in Claudin 1

Answer 7

NISCH or neonatal ichthyosis sclerosing cholangitis Syndrome

Question 8

2 major antimicrobial proteins of the skin

Answer 8

Cathelicidins abd B-defensins

Question 9

Major component of cornified envelope and is mutated in Vohnwinkel Syndrome with ichthyosis

Answer 9

Loricrin

Question 10

ABHD5

Answer 10

Chanarin-Dorfman Syndrome

Question 11

Filaggrin defect

Answer 11

Ichthyosis vulgaris

Question 12

Infant skin vs Full term newborn skin in terms of

Epidermal surface
Natural moisturizing factors
Surface pH
Sebum 
Stratum for rum water resistant
Dermal collagen fiber density
Rate of water absorption
Trans epidermal water loss

Answer 12

Infant skin

Epidermal surface: thin.
Natural moisturizing factors: low.
Surface pH: LOW
Sebum: LOW
Stratum corneum water content: high
Dermal collagen fiber density: low
Rate of water absorption: high
Trans epidermal water loss: high