Dz of epidermis, dermis and BMZ (and subQ) Flashcards

1
Q

Rare, acquired AI blistering disease of the epidermis

A

Pemphigus vulgaris

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2
Q

pemphigus vulgaris is d/t…

A

auto-ab against desmoglein 1&3

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3
Q

a positive Nikolsky’s sign is found in which disease?

A

pemphigus vulgaris

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4
Q

outcome and treatment of pemphigus vulgaris

A

fatal if not treated

immunosuppression - prednisone, azathioprine, mycophenoate mofetil, Rituximab

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5
Q

Common AD disease with mut in profilaggrin gene

A

Ichthyosis vulgaris

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6
Q

filaggrin is essential for….

A

cornified envelope

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7
Q

disease that predisposes or is associated with allergic conditions

A

ichthyosis vulgaris

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8
Q

Acquired AI dz of the dermis characterized by localized sclerosis

A

Morphea/localized scleroderma

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9
Q

what age range and gender are most commonly affected by morphea?

A

children and adult F

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10
Q

dz that presents as erythematous and indurated plauqes that slowly expand, leaving behind fibrotic or atrophic scars

A

morphea

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11
Q

what complications can be associated with morphea? which are not?

A

joint and neurologic complications

- not internal organ involvement

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12
Q

Acquired AI dz of the dermis, most common in middle-aged F

A

Systemic sclerosis

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13
Q

limited systemic sclerosis is AKA CREST. what does CREST stand for?

A
calcificus cutis
Reynaud's
Esophageal dysmotility
Sclerodactyly
Telangiecatasia
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14
Q

Dermal disease that includes pulmonary fibrosis, renal failure, GI dz, Cardiac dz

A

diffuse systemic sclerosis - widespread sclerosis of the skin

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15
Q

Acquired disorder of the SubQ fat, presenting with tender red nodules on shins

A

Erythema nodosum

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16
Q

erythema nodosum is most common in…

A

young females

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17
Q

panniculitis

A

inflammatory process involving the fat

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18
Q

most common cause of erythema nodosum

A

infections, UR strep

- also, medications, sarcoidosis, IBD, malignancy

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19
Q

outcome of erythema nodosum?

A

nodules on shins arise in crops, then slowly involute over the course of a few days/weeks

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20
Q

which type of collagen is in the lamina densa?

A

type IV

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21
Q

which type of collagen is in the sublamina densa?

A

type VII

22
Q

list 3 molecules collagen type VII binds to

A

collagens types I and IV, laminin 332/5

23
Q

most common AI bullous dermatosis

A

bullous pemphigoid

24
Q

describe the stages of bullous pemphigoid

A
  • starts with pleomorphic clinical features, e.g. highly pruritic urticaria w/o blistering
  • next, tense blisters
25
Q

bullous pemphigoid is d/t

A

auto-Ab against BP 1 and 2

26
Q

What does IIF show for Bullous Pemphigoid?

A

epidermal staining

27
Q

which as the better prognosis, bullous pemphigoid or pemphigus vulgaris?

A

BP

28
Q

what age range do bullous pemphigoid and pemphigoid vulgaris show up in?

A

elderly

29
Q

elderly pt presents with an AI dz with recurrent blistering of mucous membranes (eyes, mouth, etc.) and occasionally the skin

A

mucous membrane pemphigoid

30
Q

sequelae of MMP include…

A

blindness (20%), scars, strictures, synechiae (iris adheres to the cornea)

31
Q

MMP is d/t…

A

auto-ab to BP 180, or laminin 332/5

32
Q

MMP subtype with anti-laminin 332-Ab has an association with__________, so the doctor should ___________

A

malignancies - 30% of cases, so perform appropriate CA screening

33
Q

in a pt with MMP, IIF will stain on the….

A

epidermis - or dermis if anti laminin 332

34
Q

rare acquired AI bullous dermatosis

A

epidermolysis bullosa acquisita

35
Q

presentation of EB-acquisita

A

blistering and erosions in response to slight trauma - prevalent on mechanically stressed skin (hands, feet, elbows, knees)
- often involves the oral mucosa (hemorrhagic errosions)

36
Q

EB-acquisita is d/t…

A

anti-collagen type VII-Ab

37
Q

IIF stains where for EB-acquisita

A

dermal side

38
Q

Inherited EB is d/t

A

mutations in genes encoding BMZ proteins

39
Q

group of inherited skin fragility disorders

A

epidermolysis bullosa

40
Q

AD-inherited dz of the BMZ targeting keratins 5 and 14

A

EB-simplex

41
Q

AR-inherited dz of the BMZ targeting laminin 332 and BP180

A

Herlitz Junctional EB

42
Q

AD and AR-inherited dz of the BMZ targeting collagen type VII

A

Dystrophic EB

43
Q

subtype of EB d/t mutation in kindling 1

A

kindler syndrome

44
Q

keratohyalin granules contain

A

filaggrin

45
Q

which layer of the epidermis is keratin synthesized in

A

stratum spinosum

46
Q

lamellar granules contain

A

hydrolytic enzymes, ceramides, cholesterol and fatty acids

47
Q

what protein is responsible for thef protein cornified envelope

A

filaggrin

48
Q

what is the most abundant type of collagen in the skin

A

type I

49
Q

which corpuscles are for light touch

A

meissner’s

50
Q

which BP is cytoplasmic and belongs to the plakin family

A

BP 230

51
Q

what is BP 180 AKA?

A

collagen type XVII

52
Q

lamina densa contains primarily which two molecules?

A

collagen IV and laminin 332