Dystrophies

Question 1

Lattice

Answer 1

Amyloid deposits

Stains with Congo red

Question 2

Granular

Answer 2

Hyaline deposits
Stains with Masson trichrome
Avellino type: stellate snowflake shaped, has amyloid and hyaline deposits

Question 3

Reis buckler

Answer 3

AD
TGFBI gene
Stains with Masson trichrome

Question 4

Thiel behnke

Answer 4

AD
TGFBI gene
Honeycomb pattern

Question 5

Macular

Answer 5

AR
CHST6 gene 
Glycosaminoglycan deposits stain with colloidal iron and Alcian Blue 
Intervening haze 
Involves entire cornea 
Low levels of keratan sulfate

Question 6

Schnyder

Answer 6

AD
UBIAD1 gene 
Cholesterol and phospholipid deposits 
Stain with oil red O and Sudan black 
Check lipid panel

Question 7

Congenital stromal corneal dystrophy

Answer 7

AD
Decorin gene
Congenital bilateral clouding

Question 8

Fleck

Answer 8

AD
Pip5k3 gene
Glycosaminoglycan and lipid deposits
Congenital or early

Question 9

Posterior polymorphous corneal dystrophy

Answer 9

AD
Endothelial cells behave like epithelium
Like ICE syndrome but bilateral (stromal edema, corectopia, glaucoma)

Question 10

Meesman epithelial corneal dystrophy

Answer 10

AD
Keratin 3 or 12
Intraepithelial vesicles

Question 11

Lisch

Answer 11

X linked dominant

Band shaped feathery lesions

Question 12

Gelatinous

Answer 12

AR
Tacstd2 gene
Mulberry type, kumquat type