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EV ACVD Study > CLE (ECVD videos) > Flashcards

CLE (ECVD videos) Flashcards

1
Q

Which breeds develop VCLE

A

*Shelties
*Rough Collies
*Border collies

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2
Q

Which body regions develop VCLE

A

*Sparsely haired skin (ventral abdomen, medial thighs, flanks)
*Mucocutaneous junctions, less (lipfolds, periocular, anus)

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3
Q

Histopath pattern for VCLE

A

*Lymphocyte-rich interface dermatitis with keratinocyte vacuolation
*Confluent. vacuolation leading to intrabasal vesiculation

Lymphocytes kill keratinocytes via 1) apoptosis or 2) cell lysis and necrosis, which leads to hydropic degeneration, which leads to vacuolation

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4
Q

Chronicity of VCLE (acute, subacute, chronic)

A

Subacute

(all other forms of CLE are chronic. No acute CLE in dogs)

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5
Q

Which T cells are present in VCLE

A

CD4 and CD8

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6
Q

Clinical signs of canine juvenile dermatomyositis

A

*Scarring alopecia w/ depigmentation and focal ulcers
*Facial predominant, ear tips, tail tips, joints

NOT THE SAME AS VCLE, just the same breeds!
*Interface dermatitis but CELL POOR
*ISCHEMIC FOLLICULAR ATROPHY

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7
Q

Factor that can aggravate VCLE

A

UV light

Flares during summer, sunshine

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8
Q

Where is IgG deposited in VCLE?

A

*Epidermal BMZ
*Vessel BMZ (possible vasculitic component)
*Intracytoplasmic basal cell (because IgG is attacking a part of basal cell, cytotoxic T cells also attack this antigen on basal keratinocyte –> induce vesiculation)

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9
Q

What percent of dogs with VCLE have positive IgG against ANA in their skin?

A

0%

Cannot find ANA

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10
Q

What are the targets of IgG in VCLE on basal keratinocytes

A

Different nuclear antigens:
*SSA, SSB (also SLE in humans!)

Also
*Sm/RNP
*Jo1
*Scl70
*Sm

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11
Q

Treatment for VCLE

A

BEST DRUG = CYCLOSPORINE (active on T cells!, which attack basal keratinocytes)
*Sun avoidance
*+/- Glucocorticoids tapered for 1 month
*+/- azathioprine if still unresponsive

PENTOXI IS NOT ENOUGH, because not related to dermatomyositis

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12
Q

Prognosis VCLE

A

Complete remission in 100% with cyclosporine +/- GC

CR within 2 months in 73%

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13
Q

T or F: Most dogs need GC in addition to cyclosporine to keep their VCLE in remission

A

FALSE. 100% of dogs could be on cyclosporine alone to maintain remission of VCLE

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14
Q

Which disease causes macular and figurate erythema with superficial sloughing and erosions on lightly-haired body regions?

A

VCLE

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15
Q

What is the lupus band test?

A

Positive IgG +/- complement against epidermal BMZ, vessel BMZ, and basal keratinocytes

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16
Q

Which nuclear antigens are IgG targeting in VCLE

A

*Ro-SSA/SSB
*Other nuclear antibodies too

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17
Q

Which version of canine CLE is most similar to human SCLE

A

VCLE is a close homologue of human (vesicular) SCLE

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18
Q

Breeds ECLE

A

*German shorthaired pointers
*Vizsla

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19
Q

Chronicity of ECLE (acute, subacute, chronic)

A

Chronic

(VCLE is only subacute form; all others are chronic)

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20
Q

Sex overrepresented for ECLE

A

Females (2.1:1)

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21
Q

Age for ECLE

A

Young! 10 month median

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22
Q

Lesion distribution ECLE

A

Start on head, move to the back

Muzzle, pinnae, head –> dorsum

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23
Q

Lesion type ECLE

A

Erythema, scaling (follicular casts), alopecia

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24
Q

Mode of inheritance of ECLE

A

Autosomal recessive

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25
Q

Gene associated with ECLE

A

UNC93B1 on chromosome 18

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26
Q

Function of UNC93B1

A

Function: Chaperone/Repressor for many TLRs

With ECLE mutation: Continuous activation of TLRs by self and exogenous nucleic acids!

Hyperactivation of innate immune system –> IFN –> autoimmunity

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27
Q

When syntenin-1 is bound to UNC93B1, what is the state of Myd88?

A

Myd88 is inactive when syntenin-1 is bound (from UNC93B1)

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28
Q

When syntenin-1 is ABSENT from UNC93B1, what is the state of Myd88?

A

Persistent activation of Myd88 –> hyperactive innate immune system. Self activation of TLR7/Myd88 –> autoimmunity

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29
Q

Which signaling pathways are increased in ECLE? (3)

A

*JAK-STAT
*Interferon signaling
*Innate immune response

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30
Q

Why does Apoquel work for ECLE?

A

Because increased JAK-STAT pathway signaling in ECLE

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31
Q

T or F: Sebaceous adenitis in vizsla may actually be a manifestation of ECLE

A

True

Erythema, alopecia, scale, follicular casts

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32
Q

What are the concurrent systemic signs seen in ECLE? (6)

A

*Thrombocytopenia
*Pain/lameness
*Hunch back
*Infections
*Infertility
*Lymphadenopathy

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33
Q

Which cells are targeted by T cells in ECLE

A

*Basal keratinocytes
*Sebaceous glands

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34
Q

Which form of CLE has an associated sebaceous adenitis

A

ECLE

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35
Q

Which type of T cell is present in ECLE

A

Cytotoxic γδ T cells

36
Q

Where can you find IgG in ECLE

A

*Epidermal BMZ
*Hair follicle membrane
*Sebaceous gland membrane

37
Q

Is ECLE a true SLE?

A

YES, with time

Develop + ANA with AGE

38
Q

Prognosis ECLE

A

Poor. 44% euthanized, 31% achieve CR

39
Q

Most effective ECLE therapy

A

High dose glucocorticoids + cytotoxic drug combo to induce remission

Other reported tx: mycophenolate

40
Q

Which genes are overexpressed in ECLE

A

IFNg

Decreases with successful therapy

41
Q

Types of CLE that responds to Apoquel (oclacitinib)

A

*MCLE (100%)
*ECLE (100%)
*FDLE (75%)

0.45mg/kg BID

42
Q

Breed MCLE

A

*GSD
*Belgian Shepherd

43
Q

Sex MCLE

A

Females

Female:Male
1.7:1

44
Q

Age MCLE

A

Median 6 years

45
Q

Clinical lesions MCLE

A

*Erosions, ulcers (perianal, perigenital)
*Pain (+/- pruritus?)
*Dyschezia
*Dysuria

46
Q

Lesion distribution MCLE

A

*Perianal
*Perigenital
*Perioral

Less common:
*Periocular
*Perinasal

MOST DOGS have lesions at 2-3 MC regions

47
Q

Do MCLE lesions typically scar?

A

NO

48
Q

MCLE vs MMP similarities

A

*Both affect GSD, near MC junctions

49
Q

MCLE vs MMP differences

A

*MMP affects lip, but not surrounding skin
*MCLE affects perioral skin, but not lip mucosa itself

*MCLE: erosions/exudation
*MMP: ulcers, scars, blister formation

50
Q

Histopath MCLE

A

*Lymphocyte-rich interface dermatitis
*Thick, irregular BMZ (immune complex deposition)

51
Q

What type of Ig do you find at BMZ in MCLE

A

Mostly IgG (83%) –> Positive “lupus band test”

Also IgM (44%), C3 (33%), IgA (17%)

52
Q

Treatments MCLE

A

*Oral glucocorticoid
*Cyclosporine
*Doxy/niacinamide

53
Q

Prognosis MCLE

A

Good!
CR within 3 months

54
Q

T or F: spontaneous remission can occur in MCLE

A

False.

Usually, need lifelong meds

55
Q

Treatment with fastest time to CR in MCLE

A

Oral glucocorticoids

(1 month vs 2 months w/o)

56
Q

How do you differentiate MCLE from mucocutaneous pyoderma

A

No response to topical/oral antibiotics

57
Q

T or F: recurrence/relapses are common in MCLE if you taper immunosuppressive medication

A

True

58
Q

Clinical signs of FDLE

A

*Depigmentation
*Erythema
*Scarring
*Loss of architecture
*Crusting
*Alopecia

59
Q

Main differential diagnosis for FDLE

A

MMP

(Can affect nose, cause scarring)

(Less likely, nasal PV)

60
Q

Histopath for FDLE

A

*Lymphocytic interface dermatitis w/BMZ thickening, keratinocyte damage

Lymphocytes and plasma cells are NORMAL in mucosa, which are not present on haired skin. Can make lymphocytic interface and basal cell apoptosis harder to see

61
Q

T or F: lymphoplasmacytic lichenoid dermatitis is specific to FDLE

A

FALSE.

NONSPECIFIC lesion of mucosal and MC inflammation!!!

62
Q

Treatment FDLE

A

*Doxy/niacinamide
*Partial improvement with tacrolimus

63
Q

Overrepresented breeds GDLE

A

*Chinese crested dogs (lack of hair, more UV light exposure)
*Labrador retrievers

64
Q

Sex GDLE

A

Equal M:F

65
Q

Age GDLE

A

Older. 9 year median

66
Q

Body distribution GDLE

A

*Lateral thorax
*Dorsum
*Neck

*Abdomen
*Proximal limbs

67
Q

Clinical lesions GDLE

A

*Polycyclic plaques with central atrophic scarring
*Pigmentation changes
*Variable scaling
*Reticulated hyperpigmentation (raised if active, flat if inactive)
*MC junction involvement

Less common:
*Deep erosions, ulcers
*Hypertrophic crusting, scaling
*Scarring alopecia

68
Q

Histopath GDLE

A

Lymphocyte rich interface dermatitis

69
Q

Which Ig are present at Dermo-Epidermal junction for GDLE

A

*IgG (90%)
*IgA (80%)

70
Q

What is UNIQUE about GDLE deposits of immunoreagents

A

IgG AND IgA!!

71
Q

Can GDLE become SLE?

A

Yes, reported in 1 dog

72
Q

Does GDLE have + ANA in serum?

A

Yes, 88% dogs

But most dogs do NOT progress to SLE

73
Q

Which forms of CLE have reported SLE later?

A

ECLE
GDLE (not typical though!!)

74
Q

Does GDLE have spontaneous remission reported

A

No

75
Q

Treatment GDLE

A

*Glucocorticoids + Cyclosporine (PR)
*Doxycycline/Niacinamide (n=1, but CR)
*Tacrolimus + hydroxychloroquine

76
Q

Do lesions usually recur with medication taper for GDLE?

A

Yes

77
Q

What is unique about the immunopathology of GDLE?

A

*Positive low titers of ANA, without progression to SLE
*IgG and IgA antibodies

78
Q

Which form of CLE is reported in cats

A

GDLE
*DLE

79
Q

Which form of CLE is reported in donkeys

A

GDLE

80
Q

What species have GDLE been reported in

A

Dogs
Humans
Cats
Donkeys

81
Q

Which form of canine CLE is most similar to human DDLE

A

GDLE. Plaques, scarring, dyspigmentation

(more similar than FDLE)

82
Q

What are the lupus-nonspecific skin diseases?

A

*Vasculitis (lupus panniculitis?)
*Autoimmune BM disease (BSLE-1)

83
Q

Skin clinical signs of SLE

A

*Skin lesions (50-60%)
*MC ulcers + basal cell vacuolation = vasculitis?
*oral ulcers
*Hyperkeratotic paw pads

84
Q

Bullous SLE Type 1

A

SLE + EBA

85
Q

Clinical signs of SLE

A

*Fever
*Non-erosive Polyarthritis
*Proteinuria
*IMHA
*Leukopenia
*Thrombocytopenia
*Mouth ulcers
*Skin lesions (crusts, ulcers, scars)
*Internal organ involvement: thyroid gland, spleen, or kidneys.

86
Q

UV-A vs UV-B differences

(relevant bc UV radiation can trigger lupus)

A

UV-A = longer waves, affects dermis, causes reactive oxygen species

UV-B = shorter waves, affects epidermis, causes DNA breakage

Both damage basal keratinocytes

EV ACVD Study (52 decks)

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