Ch 4 Disorders of Keratinization Flashcards

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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
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2
Q

X Linked Recessive Icthyosis

A
  • XLR (Males)
  • Steroid Sulphatase
  • Associated with Kallman Syndrome
  • Shows up soon after birth
  • Tx is topical
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3
Q

Colloidon Baby

A
  • Not a diagnosis
  • Seen with Lamellar and Non-Bullous Icthyosis
  • Smooth stratum corneum, commonly ectropion
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4
Q

Lamellar Icthyosis and Non-Bullous icthyosiform eryhtroderma

A
  • AR
  • Colloidon baby
  • Most commonly mutations in transglutaminase 1 (Cross links keratin)
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5
Q

Epidermolytic Hyperkeratosis (Bullous icthyosiform eryhtromderma)

A
  • AD
  • Blisters worse in flexures
  • Crust often infected
  • Thickened Granular layer
  • Keratin 1 and 10
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6
Q

Harlequin Fetus

A

-ABCA12

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7
Q

Other Icthyosis

A
  • Rufman - Phytain acid peripheral neuropethy
  • Rud - Epilepsy
  • Netherton - Hair
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8
Q

Acquired

A

-Commonly seen with hodgkins

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9
Q

Keratosis Pilaris

A

-Fillagrin, seen with Icthyosis Vulgaris

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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
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11
Q

Keratoderma Palms and Soles

A
  • Generally from keratin mutations

- 9, 16, 11

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12
Q

Acquired Keratoderma

A

-Pitaryasis Rubris Pilaris
-Lichen Planus
Keratoderma Climatcticum

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13
Q

Knuckle Pads

A

Fibromatous hyperkeratotic on back of hands

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14
Q

Callous and Corn

A
  • Callous does not have keratin horn in center

- Corn has kertain in center and painful

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