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Skin > Norton Part 8 > Flashcards

Norton Part 8 Flashcards

1
Q

what is the most common and most severe type of pemphigus (80% of cases)

A

pemphigus vulgaris

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2
Q

pemphigus vulgaris typically affects what age groups

A

30-50

men and women evenly

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3
Q

pemphigus vulgaris involves what sites

A

oral ulcers

scalp, face, axillae, groin, points of pressure

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4
Q

what causes pemphigus vulgaris

A

IgG autoantibodies against demogleins 1 &3 in desmosomes

in epidermis and mucosal epithelium

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5
Q

what is a postive nikolsky sign and what is it seen in

A

pressure on blister causes lateral spread of lesion

pemphigus vulgaris

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6
Q

what is acantholysis

A

lysis of intercellular adhesions connecting squamous epithelial cells

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7
Q

a net-like pattern of intercellular IgG deposits on fluorescence is seen in what

A

pemphigus vulgaris

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8
Q

what is seen histologically in pemphigus vulgaris

A

acantholysis
single later of intact basal cells forms the blister base (row of tombstones)
superficial lymphocytic inflammatory infiltrate in dermis

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9
Q

histologically a row of tombstones is seen in what

A

pemphigus vulgaris

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10
Q

pemphigus vegetans is associated with what disorder

A

ulcerative colitis

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11
Q

what is seen in pemphigus vegetans

A

large, moist, verrous, vegetating plaques rather than blisters
oral lesions common

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12
Q

pemphigus foliaceus has what target antigen

A

Dsg 1

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13
Q

what is seen in pemphigus foliaceus

A

superficial, subcorneal blister

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14
Q

what are the target antigens in bollous pemphigoid

A

BPAg1 and BPAg2 in the hemidesmosome

only BPAg2 is known to cause blisters

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15
Q

bollus pemphigoid is typically found at what sites of what patients

A

elderly individuals
inner aspects of thighs, flexor surfaces of forearms, axillae, groin and lower abdomen
(oral lesions not as common as pemphigus vulgaris)

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16
Q

what is seen histologically in bollus pemphigoid

A

subepidermal, nonacantholytic blisters
early perivascular infiltrate of lymphocytes
eosinophils typically present in blister cavity
peripheral eosinophilia and elevated serum IgE often present

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17
Q

acantholytic blisters are seen in what

A

pemphigus vulgaris

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18
Q

linear deposition of C3 and IgG along dermoepidermal junction seen under direct immunofluorescence is seen in what

A

bollus pemphigoid

19
Q

what type of lesion is seen in bollous pemphigoid

A 
sturdy blisters (roof is full thickness of epidermis)
usually 2cm (up to 4-8)
do not rupture as easily as in pemphigus
without infection will heal without scarring
20
Q

dermatitis herpetiformis is seen in what patients

A

20-60 years old

2:1 M:F ratio

21
Q

dermatitis herpetiformis is due to what

A

IgA autoantibodies to transglutaminases bind to TG in gut and cross-react with reticulin in fibrils in skin

22
Q

dermatitis herpetiformis is associated with what

A

HLA-B8
HLA-DR
HLA-DQ

23
Q

the lesions in dermatitis herpetiformis appear how

A

symmetrically grouped lesions
papules and plaques progressing to vessivles and bullae
very pruritic

24
Q

dermatitis herpetiformis is associated with what disorder

A

Celiac disease

25
Q

dermatitis herpetiformis is typically found at what sites

A

buttocks, elbows, knees, scapular areas

26
Q

what is seen histologically in dermatitis herpetiformis

A

microabscesses at dermal papillae
subepidermal blister
neutrophil microabscesses and fibrin deposition in tips of dermal papillae
basal cells overlying tips show dermoepidermal separation eventually coalescing to form blister

27
Q

how is bollous pemphigoid diagnosed

A 
skin biopsy
antiendomysian antibodies (specific for TG)
endoscopy with biopsy showing blunting of villi in small bowel
28
Q

epidermolysis bullosa is what

A

a group of disorder caused by inherited defects in proteins that lend stability to the skin

29
Q

what is common in all forms of epidermolysis bullosa

A

tendency to form blisters at points of pressure, rubbing, trauma

30
Q

epidermolysis bullosa simplex is caused by what

A

mutation in either gene encoding keratin 14 or 5 which form a functional keratin fiber resulting in basal cell layer defects

31
Q

junctional epidermolysis bullosa is characterized by what

A

blisters formed from separation of lamina lucida

32
Q

dystrophic epidermolysis bullosa is characterized by what

A

blisters below lamina densa
defect in collagen 7
scar formation on healing

33
Q

non-herlitz junctional epidermolysis bulls is characterized by what

A

laminin V(beta)3 defect

34
Q

what is seen histologically in epidermolysis bullosa

A 
fibrin deposition in the floor of the blister cavity
some cases may show neutrophils within the papillary dermis (others are indistinguishable from bolos pemphigoid)
dermal fibrosis (scar) may be present in dermis
35
Q

porphyria cutanea tarda is seen in what patients

A

30-50 yrs
females on OCPs
alcohol use
associated with hepatitis C

36
Q

what is the efficiency in prophyria cutanea tarda and what does that lead to

A 
uroporphyrinogen decarboylase (UROD)
excessive accumulation of porphyrins
37
Q

what is type I porphyria cutanea tarda

A

acquired disease via exposure to drugs/chemicals

hepatic UROD is inhibited

38
Q

what is Type II prophyria cutanea tarda

A

autosomal dominant

deficient in RBCs and fibroblasts

39
Q

what does the accumulation of porphyrins do in porphyria cutanea tarda

A

makes skin photosensitive

40
Q

what is seen in prophyria cutanea tarda

A

tense subepidermal bulla on normal appearing skin in sun exposed areas
mat also get hypertrichnsis on face

41
Q

what exacerbates porphyria cutanea tarda

A 
iron
alcohol
smoking
estrogens
hepatitis C
HIV
halogenated hydrocarbons
42
Q

how is porphyria cutanea tarda diagnosed

A

test for uroporphyrin in urine

orange color under woods lamp

43
Q

what fluoresces orange under a woods lamp

A

porphyria cutanea tarda

44
Q

what histological features are seen in porphyria cutanea tarda

A

subepidermal vesiculation
aural skin with compact orthokeratosis
solar elastosis
protuberance of rigid dermal papillae into blister cavity
thickening of walls of superficial dermal vessels

Skin (12 decks)

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