ACVD Exam > Structure and Function - Cornification disorders > Flashcards

Structure and Function - Cornification disorders Flashcards

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1
Q

The dry form of canine seborrhea

A

Seborrhea sicca

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2
Q

The greasy form of canine seborrhea

A

Seborrhea oleosa

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3
Q

Broad disease categories that can cause canine seborrhea (a defect in keratinization with increased scale formation)

A
  • primary/genetic
  • inflammatory conditions
  • endocrine diseases
  • nutritional deficiencies
  • neoplasia
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4
Q

Actions of benzoyl peroxide

A
  • degreaser
  • keratolytic
  • follicular flushing
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5
Q

Dog breeds prone to primary seborrhea, an inherited cellular keratinization defect

A
  • America cocker spaniel
  • English springer spaniel
  • WHWT
  • Bassett hounds
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6
Q

In primary seborrhea is epidermal turnover time increased or decreased?

A

decreased (8 days vs normal 21-22 days)

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7
Q

In WHWT is the mode of inheritance for primary seborrhea considered autosomal recessive or autosomal dominant?

A

AR - autosomal recessive

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8
Q

Clinical signs of primary seborrhea

A
  • Mild flaking/dullness of coat at 10w
  • Seborrhea worsens at 12-16m of age
  • Ceruminous otitis, dull coat, greasy/malodorous skin, follicular casts (varies based on affected breed) +/- pruritus +/- Malassezia
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9
Q

Primary seborrhea histologic findings

A

non infected flaky or greasy areas, hyperplastic superficial perivascular dermatitis, orthokeratosis and/or hyperkeratosis

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10
Q

Breed most commonly diagnosed with Vitamin-A responsive dermatosis

A

Cocker Spaniels
Other reported breeds: Labradors, mini Schnauzers, Gordon setters etc

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11
Q

Clinical signs of Vitamin-A responsive dermatosis

A
  • Marked follicular plugging
  • hyperkeratotic plaques
  • dry, disheveled, easily epilated hair coat
  • +/- ceruminous otitis
  • +/- rancid odor
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12
Q

Key histologic feature of Vitamin-A responsive dermatosis

A

Disproportionate follicular orthokeratotic hyperkeratosis

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13
Q

Recommended dose of Vitamin-A for treatment of vit-A responsive dermatosis

A

Vitamin A 10,000U per day (max safe dose 1200 U/kg/day)
(retinol has also been used historically)

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14
Q

Epidermloytic ichthyosis is well characterized in what breed of dog?

A

Norfolk terriers

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15
Q

A defect in what gene is responsible for the development of epidermolytic ichthyosis in Norfolk terriers?

A

KRT10 gene (autosomal recessive splice mutation)

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16
Q

Histologic findings of epidermolytic ichthyosis d/t KRT10 defect

A

acanthosis (thickened stratum spinosum) with hyperkeratosis, cytolysis in granular layer, and coarse keratohyalin granules
(severe cases resulting in skin fragility and erosion with pressure are reported)

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17
Q

Inheritance of congenital ichthyosis in Golden Retrievers, American Bulldogs, Great Danes, Jack Russell Terriers, and Cavalier King Charles Spaniels is autosomal recessive or autosomal dominant?

A

Autosomal recessive

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18
Q

Gene associated with type I ichthyosis in Golden retrievers

A

PNPLA1

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19
Q

Gene associated with type II ichthyosis in Golden retrievers

A

ABHD5

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20
Q

Clinical signs of ARCI (autosomal recessive congenital ichthyosis) in Golden Retrievers

A

Large soft, white-to-grey adherent scale and ventral hyperpigmentation
(usually diagnosed by 1y of age)

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21
Q

Gene associated with ARC Ichthyosis in American Bulldogs

A

NIPAL-4

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22
Q

Clinical signs of ARCI in American Bulldogs

A

Generalised large white scale, erythematous and roughened skin with adherent brown scale on abdomen +/- Malassezia +/- pododermatitis +/- otitis

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23
Q

Gene associated with ARCI in Great Danes? What does that gene encode?

A
  • SLC27A4
  • FATP4 (fatty acid transporter 4)
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24
Q

Prognosis for ARCI in Great Danes?

A

Guarded - often euthanized

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25
Clinical signs of ARCI in Great Danes
- Severe greasy scaling with marked wrinkling of the skin of the face and extremities - Severely affected from birth - often euthanized
26
General histologic features of non-epidermolytic ARCI ichthyosis
- Moderate to severe, compact lamellar epidermal orthokeratotic hyperkeratosis - Mild, chronic, pleocellular superficial perivascular dermatitis sometimes with eosinophils
27
Additional histologic feature of ARCI in Great Danes
accumulation of eosinophilic material (glycosaminoglycan) in hair follicle
28
ARCI of Jack Russell Terriers tends to have a mild, moderate, or severe clinical presentation?
SEVERE
29
Classic appearance of scale in JRT with ARCI?
Parchment paper
30
Mutation in this gene is responsible for ARCI in JRTs
TGM1 (tranglutaminase 1 plays an important role in the formation of the cornified envelope)
31
Genetic mutation of what gene is responsible for ARCI in Cavaliers?
FAM83H
32
Dermatologic clinical signs of ARCI caused by mutation in FAM83H in Cavaliers?
Scaling, roughened pelage (haircoat), footpad hyperkeratosis, onychodystrophy
33
The gene that causes ARCI in Cavaliers also impacts what other part of the body?
Eyes -> KCS
34
Palmoplantar keratoderma is caused by a mutation in what gene in Dogues de Bordeauxs?
KRT16
35
Where is the KRT16 gene expressed?
- paw pads - nasal planum
36
Clinical signs of palmoplantar keratoderma of Dogues de Bordeauxs
- Reduced tensile strength in affected areas -> increased hyperkeratosis in weight bearing areas - Fissures and cracks of the paw pads and nasal planum
37
Histologic findings in palmoplantar keratoderma of Dogues de Bordeauxs
- SC expanded by tremendous orthokeratotic hyperkeratosis and marked parakeratosis at tips of villae
38
Treatment of palmplantar keratoderma of Dogues de Bordeauxs?
- daily propylene glycol soaks - dremel
39
A condition that is clinically similar to palmoplantar keratoderma of Dogues de Bordeauxs has been reported in Irish Terriers and Kromfohrlanders with a mutation in what gene?
FAM83G
40
Footpad hyperkeratosis in a Rottweiler has been found to be associated with a frameshift mutation in what gene?
DSG1 (compensatory hyperkeratosis)
41
Hereditary nasal parakeratosis is primarily reported in what 2 breed of dog?
- Labradors - Greyhounds
42
A mutation in what gene is responsible for hereditary nasal parakeratosis in Labs and Greyhounds? What does that mutation cause?
- SUV39H2 - decreased expression of loricrin
43
Mode of inheritance of hereditary nasal parakeratosis in Labs and Greyhounds
Autosomal recessive
44
CHILD syndrome's full name
Congenital hemidysplasia with ichthyosiform erythroderma and limb deficits
45
What is CHILD syndrome in humans?
rare X-linked dominant disorder that is manifested as a combination of unilateral limb deficits with hyperkeratotic plaques along with neurological and developmental disorders
46
In CHILDs syndrome, there is a metabolic error in the generation of what lipid?
Cholesterol
47
CHILDs syndrome is caused by mutations in what protein
NSDHL *results in a failure to convert lanosterol to cholesterol, which leads to both an accumulation of toxic sterol precursors and a deficiency of cholesterol in SC lamellar bilayers*
48
NSDHL variant mutations have been reported in what dog breeds?
- Labrador retriever - Chihuahua
49
A novel form of ichthyosis in German Shepherd Dogs is caused by a defect in what gene? What does that gene impact?
- ASPRV1 - filaggrin processing
50
Ichthyosis fetalis in Chianina cattle is caused by a mutation in what gene?
ABCA12 (H1935R) - this disease tends to lethal in calves
51
Naked foal syndrome in Akhal-Teke horses is caused by a defect in what gene?
ST14 - affects filaggrin processing (this disease is almost always fatal)
52
Differentials for nasodigital hyperkeratosis
- hereditary nasal hyperkeratosis (Labs) - anatomically reduced friction (d/t conformation) - Distemper - Leishmaniosis - PF - Drug reaction - Systemic lupus erythematosus - Zinc responsive dermatosis - Hepatocutaneous syndrome - Cutaneous lymphoma
53
Feline chin acne is an idiopathic disorder affecting what follicular process?
Follicular keratinization
54
Differentials for feline chin acne
- demodicosis - dematophytosis - Malassezia dermatitis
55
Treatment that most reliably controls feline chin acne?
Mupirocin (>95% response)
56
What disease should you be concerned for in an older cat with non-pruritic exfoliative dermatitis +/- leukotrichia?
thymoma associated exfoliative dermatitis
57
Key histologic finding in skin biopsies from cats with thymoma associated exfoliative dermatitis
interface dermatitis composed of mainly CD3+ lymphocytes and varying amounts of mast cells and plasma cells
58
Differentials for exfoliative dermatitis in cats
- FeLV/FIV dermatitis - SLE, drug reaction - demodicosis - cutaneous lymphoma
59
Pinnal margin seborrhea with follicular casting is most common in what dog breed?
Dachshunds
60
Management of ear margin dermatosis
- antiseborrheic shampoos (eg, sulfur, salicylic acid, benzoyl peroxide) - keratolytic products such as salicylic acid gel - topical moisturizers - vitamin A - essential fatty acids. - antiinflammatory drug (GCs, pentoxy, etc)
61
Zinc-responsive dermatosis causes what type of hyperkeratosis?
Parakeratotic
62
Zinc responsive dermatosis syndrome I predominantly occurs in breed group?
Northern/Nordic breeds (Siberian husky, Alaskan malamute, Samoyed) *likely an inherited disorder*
63
Clinical signs of zinc responsive dermatosis
thick, adherent, crusted, hyperkeratotic plaques, often affecting the face
64
A colloquial name for Zinc Responsive Dermatosis Type II
"generic dog food dermatosis"
65
what is Zinc Responsive Dermatosis Type II is caused by?
feeding of imbalanced diets with a relative deficiency in zinc
66
Treatment of zinc responsive dermatosis?
2–3 mg/kg p.o. daily of elemental zinc
67
Localized parakeratotic hyperkeratosis of Boston Terrier (and maybe Frenchies?) has been shown to be responsive to what therapy in some affected dogs?
zinc supplementation
68
A mutation in what gene causes ichthyosis in Shar Peis?
KRT1
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