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Vet Derm Review > Desmosomes - Croatia > Flashcards

Desmosomes - Croatia Flashcards

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Dermatology Board Prep flashcards
1
Q

Both Darier Disease and Hailey-Hailey Disease have what mode of inheritance?

A

autosomal dominant

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2
Q

Autoantibodies against periplakin can result in what clinical disease?

A

paraneoplastic pemphigus

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3
Q

Why are oral lesions not a feature of pemphigus foliaceus?

A

desmocollin-1 is not present in the oral mucosa

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4
Q

Why do autoantibodies against desmolgein-3 or desmocollin-3 result in more severe lesions?

A

These proteins are expressed in keratinocytes of stratum spinosum & stratum basale

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5
Q

What is the major antigen of pemphigus foliaceus in dogs?

A

desmocollin-1

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6
Q

Where are the most common areas to be affected with canine pemphigus foliaceus?

A

nasal planum and/or dorsal muzzle; trunk, pinnae, footpads

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7
Q

ExpA and ExpB toxins of S. pseudintermedius target what structure in the canine desmosome?

A

DSG-1

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8
Q

Is desmoglein-1 present in the superficial epidermis (corneum/granulosum) or deep epidermis (spinosum/basale)?

A

Both – but more present in superficial epidermis

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9
Q

What species of dermatophytes has been demonstrated to cause superficial acantholytic pustular dermatophytosis? In what animal species has this been reported?

A

Trichophyton spp., dogs and horses

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10
Q

In superficial pustular dermatophytosis, what structures of the epidermis are invaded by dermatophytes?

A

stratum corneum, follicular infundibula, subcorneal pustules

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11
Q

To what does desmoglein-3 bind?

A

amino-terminus is heterophilic binds to DSC1, DSC2, DSC3; carboxy-terminus: plakophilin-3, plakoglobin

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12
Q

T/F: Trichograms from animals suspected to have superficial pustular dermatophytosis are the best sample for fungal culture.

A

False – do NOT invade the hair shaft, need the scale (i.e. the stratum corneum)

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13
Q

What is the target autoantigen in pemphigus foliaceus in dogs?

A

DSC-1 (major), DSG-1 (minor) - dogs

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14
Q

What are the two main members of the desmosomal cadherin family?

A

desmogleins and desmocolins

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15
Q

T/F: Many dogs with pemphigus foliaceus also suffer from a concurrent autoimmune disease.

A

True - up to 19% have a concurrent autoimmune disease (IMHA, ITP, KCS, thyroiditis)

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16
Q

T/F: The exfoliatin toxin of both S. aureus and S. hyicus cleave canine DSG-1.

A

False – different attachment site in the dog

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17
Q

Histological features of Darier disease?

A

acanthosis with orthokeratotic and parakeratotic hyperkeratosis; marked, diffuse, multifocal areas of acantholysis of the lower and middle portions of the epidrmis and follicular outer root sheet; acantholytic dyskeratotic keratinocytes (corps ronds) may be visible

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18
Q

T/F: Gram-positive cocci are usually present in the subcorneal pustules seen with exudative epidermitis.

A

True– though you usually need special stains to see it

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19
Q

Median age of onset for Pemphigus foliaceus in dogs? Cats? Horses? Small Ruminants?

A

Dogs: middle-aged (6 years), Cats: middle aged (6.5 years), Horses: 7 years, Small ruminants: young (median 1.5 years)

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20
Q

To what does plakophilin-1 bind?

A

cytoplasmic segment of desmogleins and desmocollins; desmoplakin, plakoglobin, keratins

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21
Q

What the clinical signs of pemphigus vegetans?

A

flaccid blister –> widespread erosions –> heal with excessive proliferation –> verrucous plaques, especially in areas of friction

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22
Q

To what does desmoplakin-1/2 bind?

A

amino-terminus: plakoglobin, plakophilins, DSC1, DSG1; carboxy-terminus: keratins

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23
Q

T/F: Desmogleins only bind to other desmogleins.

A

False - desmogleins DO NOT bind to each other, they ONLY bind to desmocolins

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24
Q

What type of T cells help naïve B cells differentiate into plasma cells? Through what cytokines?

A

Th2 helper cells; IL-5, IL-13, IL-6, IL-10

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25
Q

What are the primary lesions of pemphigus vulgaris?

A

flaccid vesicle rapidly progressing to an erosion, crusts can develop over lesions at mucocutaneous junctions or haired skin

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26
Q

Mutations in the gene that encodes for plakophilin-1 (PKP1) has been described in what breed?

A

Chesapeake Bay retrievers

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27
Q

What is the target autoantigen in pemphigus vegetans (dog)?

A

DSG1

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28
Q

Do dogs or cats more commonly exhibit signs of systemic illness with pemphigus foliaceus?

A

Cats – up to 50% of cats have non-dermatological signs (lethargy, fever, anorexia), reported in ~30% of dogs; cats and dogs both can exhibit pruritus

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29
Q

What are the plakins present in the epidermis?

A

BPAG1-e (BP230), plectin, desmoplakin 1/2, envoplakin, periplakin, epiplakin

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30
Q

Clincal presentation of exfoliative superficial pyoderma

A

superficial spreading pyoderma – rapidly expanding epidermal collarettes that can coalesce to cover large areas with a polycyclic pattern; staphyloccal scalded skin syndrome – erythema with overlying scaling composed of large sheets of stratum corneum

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31
Q

What virulence factor of Staph. Aureus results in subcorneal pustules?

A

exfoliatin toxin A, B, D (proteases that can bind to and cleave DSG-1)

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32
Q

What are the typical lesions of canine Darier disease?

A

nonpruritic alopecic, scaly and crusted plaques –> hyperplastic

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33
Q

What is the target autoantigen in paraneoplastic pemphigus? In what species?

A

described in dogs & cats; autoantigen known for dogs only: DSG3, plakins (evoplakin, periplakin)

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34
Q

T/F: With insecticide-triggered pemphigus foliaceus, lesions were localized to the site of application of the product in most cases.

A

False – over 70% of cases were generalized (head, face, paws, trunk affected) in addition to site of application

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35
Q

At what age does Darier disease usually become apparent?

A

during puberty

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36
Q

T/F: The autoantigen of pemphigus vegetans is the same in dogs and people, DSG-1.

A

FALSE – DSG-1 is the target in DOGS, but DSG-3 is the major antigen in people (few cases of DSG-1 and desmocollin in people)

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37
Q

To what does plakoglobin bind?

A

desmogleins and desmocolins, desmoplakins, plakophilins, keratins

38
Q

T/F: The feet and coronary bands are usually NOT affected with exudative epidermitis in pigs.

A

False - usually bear similar lesions (erythema, brown exudation, crusting)

39
Q

What are the histological characteristics of bullous impetigo?

A

large subcorneal or intragranular pustules, containing neutrophils and variable numbers of acantholytic keratinocytes, gram+ cocci may be seen in pustules

40
Q

What body sites are affected “atypical” canine pemphigus foliaceus?

A

trunk-predominant without nasal planum/muzzle involvement

41
Q

T/F: In bullous impetigo, it is common to see spreading collarettes along with follicular papules and pustules.

A

False – these lesions do not typically coexist with lesions typical of bacterial folliculitis

42
Q

Typical clinical presentation of superficial pustular dermatophytosis

A

localized papules/pustules/scaling/crusts –> spread centrifugally to affect larger areas (expansion is a sign of proteolysis)

43
Q

What is distribution of lesions in cats with pemphigus foliaceus?

A

face (pinnae, eyelids, nasal planum/dorsal muzzle), feet (claw folds and/or footpads), perioral, mammary papillae

44
Q

Autoantibodies against desmoglein-3 can result in what clinical disease?

A

mucosal pemphigus vulgaris, mucocutaneous pemphigus vulgaris, paraneoplastic pemphigus (rare in dogs)

45
Q

To what does periplakin bind?

A

amino-terminus: envoplakin, involucrin; carboxy-terminus: keratins

46
Q

T/F: Acantholytic keratinocytes are commonly identified with exfoliative superficial pyoderma.

A

FALSE

47
Q

Non-dermatological signs in equine pemphigus foliaceus

A

pruritus (~50% of horses), pain, swelling/edema, lethargy, fever (30-50%)

48
Q

To what does desmocollin-1 bind?

A

amino-terminus heterophilic, binds to DSG1/DSG2/DSG3/DSG4, carboxy-terminus: PKP1/PKP2/PKP3 (plakophilin), PG (plakoglobin), desmoplakin

49
Q

What is the characteristic lesion of pemphigus foliaceus?

A

subcorneal pustule (rapidly progresses into an erosion, crust, scale-crust, alopecia)

50
Q

T/F: Circulating antikeratinocyte IgG has been demonstrated in dogs, cats, and horses, but not other species.

A

True –has not been demonstrated in goats and sheep with PF

51
Q

What is the major antigen of paraneoplastic pemphigus in dogs?

A

DSG-3, plakins

52
Q

What molecule is important for cadherin conformation and the strength of intermolecular adhesions?

A

calcium

53
Q

T/F: Autoreactive T cells are the primary mediator of pemphigus diseases.

A

False - they play a role, but is primarily an antibody-mediated disease

54
Q

What strains of bacteria have been associated with exudative epidermitis in pigs?

A

Staph. Hyicus, S. chromogenes, S. sciuri

55
Q

Differentials for acantholytic pustular dermatitis in the dog

A

pemphigus foliaceus, superficial pyoderma (impetigo), pustular dermatophytosis, pustular dermatitis associated with leishmaniosis

56
Q

Histopathology characteristics of pemphigus foliaceus

A

subcorneal to intragranular pustule containing individualized or clustered acantholytic keratinocytes; pustules may span several hair follicles & may extend into infundibula; neutrophils are predominant inflammatory cell type +/- eosinophils; multilayered neutrophilic or eosinophilic crusts with ghost acantholytic cells may be seen in cats & horses

57
Q

Distribution of lesions of equine pemphigus foliaceus

A

symmetrical and generalized: face (pinnae, muzzle, eyelids), extremities (distal limbs, coronary bands), neck, and/or trunk

58
Q

What are the exfoliatin-toxins of S. pseudintermedius?

A

EXPA and EXPB

59
Q

In what species has pemphigus vulgaris been described?

A

dogs, horses, cats, primates, humans

60
Q

T/F: Desmocollin-3 is the major antigen of pemphigus vulgaris in dogs.

A

False - desmoglein-3

61
Q

Suprabasal Acantholytic Epidermolysis Bullosa in cattle has what mode of inheritance?

A

autosomal recessive

62
Q

How can bacterial or fungal (dermatophyte) diseases results in acantholysis?

A

proteases degrade the proteins in the desmosomes –> resulting in acantholysis

63
Q

What are the clinical features of bullous impetigo?

A

nonfollicular flaccid or tense pustules, which rupture and become overlain with yellow crusts –> expanding epidermal collarettes

64
Q

Paraneoplastic pemphigus clinically resembles what other form of pemphigus?

A

Pemphigus vulgaris

65
Q

What is the target autoantigen in pemphigus vulgaris in the dog? Horse?

A

dog: DSG3, DSG1 (mucocutaneous form); horses: DSG3, DSG1

66
Q

Histopathology characteristics of pemphigus vegetans

A

suprabasal acantholysis, papillomatous or verrucous proliferation of epithelium; intraepithelial microabscesses containing neutrophils and eosinophils +/- apoptotic or necrotic keratinocytes

67
Q

Histopathology characteristics of pemphigus vulgaris

A

suprabasal acantholysis –> blister formation, usually free of inflammatory cells; submucosal and dermal inflammation from mucocutaneous junctions is superficial lymphomasmacytic band-like infiltrrate, which is a nonspecific inflammatory response in these anatomic locations

68
Q

What are the members of the armadillo family?

A

plakoglobin, plakophilins

69
Q

What is the distribution of lesions of pemphigus vulgaris?

A

mucosae/mucocutaneous junctions (oral cavity, nasal planum, lip margins, genitalia, anus, eyelids) & pinnae; haired skin may or may not be involved**

70
Q

Histologically, paraneoplastic pemphigus resembles what other disease processes?

A

pemphigus vulgaris: suprabasal acantholysis; erythema multiforme: apoptotic keratinocytes and mononuclear interface dermatitis; dermo-epidermal separation: bullous pemphigoid

71
Q

T/F: Paraneoplastic pemphigus can affect both mucosae/mucocutaneous junctions and haired skin

A

TRUE

72
Q

Canine Darier Disease is reported in what breed?

A

English setter

73
Q

Autoantibodies against desmoglein-1 can result in what clinical disease?

A

rare in dogs (not most common antigen)– pemphigus foliaceus, mucocutaneous pemphigus vulgaris, pemphigus vulgaris (rare)

74
Q

What is the most common pemphigus variant in people? In dogs?

A

pemphigus vulgaris in people, pemphigus foliaceus in dogs

75
Q

Why can animals with pemphigus foliaceus have thick, multilayered crusts?

A

repeated waves of pustule formation

76
Q

Autoantibodies against desmoplakin-1/2 result in what clinical disease?

A

Paraneoplastic pemphigus

77
Q

Clinical lesions of equine pemphigus foliaceus

A

intact pustules are very rarely seen; common lesions are shallow erosions, crusts, scrust-scales, and/or tufted crusts

78
Q

Differentials for acantholytic pustular dermatitis in the horse & cat

A

pemphigus foliaceus, pustular dermatophytosis, impetigo (rare)

79
Q

Autoantibodies against envoplakin can result in what clinical disease?

A

paraneoplastic pemphigus

80
Q

T/F: The autoantigen of pemphigus vulgaris is the same in dogs and humans, DSG-3.

A

True, DSG-1 is detected in a minority of dogs with PV

81
Q

What breeds of dog are over-represented with pemphigus foliaceus?

A

Chow-Chow and Akita

82
Q

Darier disease likely results from mutations in genes that encode what structures?

A

calcium pumps – impair desmosomal function

83
Q

What breeds of dog are over-represented with pemphigus vulgaris?

A

German Shepherd, collie

84
Q

Mutations in the gene that encodes for plakophilin-1 (PKP1) result in what clinical disease?

A

Ectodermal dysplasia/skin fragility syndrome (Suprabasal Epidermolysis bullosa Simplex) – recognized in one line of Chesapeake bay retrievers

85
Q

What structure is suspected to be targeted in exfoliative superficial pyoderma leading to crust formation

A

corneodesmosin

86
Q

Is desmoglein-3 present in the superficial epidermis (corneum/granulosum) or deep epidermis (spinosum/basale)?

A

both – but more present in deep epidermis (stratum basale)

87
Q

To what does desmoglein-1 bind?

A

amino-terminus is heterophilic binds to DSC1, DSC2, DSC3; carboxy-terminus: desmoplakin, plakophilins, plakoglobin, keratin-5

88
Q

T/F: A negative anti-keratinocyte IgG assay rules out a diagnosis of atypical pemphigus foliaceus.

A

False – positive result supports the diagnosis but negative does not rule it out (up to 50% can test negative)

89
Q

T/F: Subcorneal pustules with acantholytic keratinocytes and lymphocytic interface dermatitis are features of superficial pustular dermatophytosis.

A

TRUE

90
Q

Pemphigus vegetans is considered to be a variant of which other disease?

A

Pemphigus vulgaris

91
Q

Mutations in the gene that encodes for what results in lethal acantholytic epidermolysis bullosa simplex?

A

desmoplakin (DSP)

Vet Derm Review (44 decks)

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