Ch 4 Disorders of Keratinization Flashcards
1
Q
Icthyosis Vulgaris
A
- A semi dominany fillagrin (Keratohyalin Granule)
- Most obvious in major flexures
- Associated with Keratosis Pilaris
- Not usually present at birth, develops
- Co morbid with atopic eczema
- Tx-Emulsifying
2
Q
X Linked Recessive Icthyosis
A
- XLR (Males)
- Steroid Sulphatase
- Associated with Kallman Syndrome
- Shows up soon after birth
- Tx is topical
3
Q
Colloidon Baby
A
- Not a diagnosis
- Seen with Lamellar and Non-Bullous Icthyosis
- Smooth stratum corneum, commonly ectropion
4
Q
Lamellar Icthyosis and Non-Bullous icthyosiform eryhtroderma
A
- AR
- Colloidon baby
- Most commonly mutations in transglutaminase 1 (Cross links keratin)
5
Q
Epidermolytic Hyperkeratosis (Bullous icthyosiform eryhtromderma)
A
- AD
- Blisters worse in flexures
- Crust often infected
- Thickened Granular layer
- Keratin 1 and 10
6
Q
Harlequin Fetus
A
-ABCA12
7
Q
Other Icthyosis
A
- Rufman - Phytain acid peripheral neuropethy
- Rud - Epilepsy
- Netherton - Hair
8
Q
Acquired
A
-Commonly seen with hodgkins
9
Q
Keratosis Pilaris
A
-Fillagrin, seen with Icthyosis Vulgaris
10
Q
Keratosis Follicularis (Dariers Disease)
A
- AD ATPA2
- Brown to yellow warty papules
- Mid Teens, seborrheic areas (light)
- Cobblestone
- Pits on palms and soles
- longitudinal pink nail dystrophy
11
Q
Keratoderma Palms and Soles
A
- Generally from keratin mutations
- 9, 16, 11
12
Q
Acquired Keratoderma
A
-Pitaryasis Rubris Pilaris
-Lichen Planus
Keratoderma Climatcticum
13
Q
Knuckle Pads
A
Fibromatous hyperkeratotic on back of hands
14
Q
Callous and Corn
A
- Callous does not have keratin horn in center
- Corn has kertain in center and painful