Dz of epidermis, dermis and BMZ (and subQ)

Question 1

Rare, acquired AI blistering disease of the epidermis

Answer 1

Pemphigus vulgaris

Question 2

pemphigus vulgaris is d/t…

Answer 2

auto-ab against desmoglein 1&3

Question 3

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

Answer 3

pemphigus vulgaris

Question 4

outcome and treatment of pemphigus vulgaris

Answer 4

fatal if not treated

immunosuppression - prednisone, azathioprine, mycophenoate mofetil, Rituximab

Question 5

Common AD disease with mut in profilaggrin gene

Answer 5

Ichthyosis vulgaris

Question 6

filaggrin is essential for….

Answer 6

cornified envelope

Question 7

disease that predisposes or is associated with allergic conditions

Answer 7

ichthyosis vulgaris

Question 8

Acquired AI dz of the dermis characterized by localized sclerosis

Answer 8

Morphea/localized scleroderma

Question 9

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

Answer 9

children and adult F

Question 10

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

Answer 10

morphea

Question 11

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

Answer 11

joint and neurologic complications

- not internal organ involvement

Question 12

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

Answer 12

Systemic sclerosis

Question 13

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

Answer 13

calcificus cutis
Reynaud's
Esophageal dysmotility
Sclerodactyly
Telangiecatasia

Question 14

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

Answer 14

diffuse systemic sclerosis - widespread sclerosis of the skin

Question 15

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

Answer 15

Erythema nodosum

Question 16

erythema nodosum is most common in…

Answer 16

young females

Question 17

panniculitis

Answer 17

inflammatory process involving the fat

Question 18

most common cause of erythema nodosum

Answer 18

infections, UR strep

- also, medications, sarcoidosis, IBD, malignancy

Question 19

outcome of erythema nodosum?

Answer 19

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

Question 20

which type of collagen is in the lamina densa?

Answer 20

type IV

Question 21

which type of collagen is in the sublamina densa?

Answer 21

type VII

Question 22

list 3 molecules collagen type VII binds to

Answer 22

collagens types I and IV, laminin 332/5

Question 23

most common AI bullous dermatosis

Answer 23

bullous pemphigoid

Question 24

describe the stages of bullous pemphigoid

Answer 24

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

Question 25

bullous pemphigoid is d/t

Answer 25

auto-Ab against BP 1 and 2

Question 26

What does IIF show for Bullous Pemphigoid?

Answer 26

epidermal staining

Question 27

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

Answer 27

BP

Question 28

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

Answer 28

elderly

Question 29

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

Answer 29

mucous membrane pemphigoid

Question 30

sequelae of MMP include...

Answer 30

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

Question 31

MMP is d/t...

Answer 31

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

Question 32

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

Answer 32

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

Question 33

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

Answer 33

epidermis - or dermis if anti laminin 332

Question 34

rare acquired AI bullous dermatosis

Answer 34

epidermolysis bullosa acquisita

Question 35

presentation of EB-acquisita

Answer 35

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

Question 36

EB-acquisita is d/t...

Answer 36

anti-collagen type VII-Ab

Question 37

IIF stains where for EB-acquisita

Answer 37

dermal side

Question 38

Inherited EB is d/t

Answer 38

mutations in genes encoding BMZ proteins

Question 39

group of inherited skin fragility disorders

Answer 39

epidermolysis bullosa

Question 40

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

Answer 40

EB-simplex

Question 41

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

Answer 41

Herlitz Junctional EB

Question 42

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

Answer 42

Dystrophic EB

Question 43

subtype of EB d/t mutation in kindling 1

Answer 43

kindler syndrome

Question 44

keratohyalin granules contain

Answer 44

filaggrin

Question 45

which layer of the epidermis is keratin synthesized in

Answer 45

stratum spinosum

Question 46

lamellar granules contain

Answer 46

hydrolytic enzymes, ceramides, cholesterol and fatty acids

Question 47

what protein is responsible for thef protein cornified envelope

Answer 47

filaggrin

Question 48

what is the most abundant type of collagen in the skin

Answer 48

type I

Question 49

which corpuscles are for light touch

Answer 49

meissner's

Question 50

which BP is cytoplasmic and belongs to the plakin family

Answer 50

BP 230

Question 51

what is BP 180 AKA?

Answer 51

collagen type XVII

Question 52

lamina densa contains primarily which two molecules?

Answer 52

collagen IV and laminin 332