Norton Part 8

Question 1

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

Answer 1

pemphigus vulgaris

Question 2

pemphigus vulgaris typically affects what age groups

Answer 2

30-50

men and women evenly

Question 3

pemphigus vulgaris involves what sites

Answer 3

oral ulcers

scalp, face, axillae, groin, points of pressure

Question 4

what causes pemphigus vulgaris

Answer 4

IgG autoantibodies against demogleins 1 &3 in desmosomes

in epidermis and mucosal epithelium

Question 5

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

Answer 5

pressure on blister causes lateral spread of lesion

pemphigus vulgaris

Question 6

what is acantholysis

Answer 6

lysis of intercellular adhesions connecting squamous epithelial cells

Question 7

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

Answer 7

pemphigus vulgaris

Question 8

what is seen histologically in pemphigus vulgaris

Answer 8

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

Question 9

histologically a row of tombstones is seen in what

Answer 9

pemphigus vulgaris

Question 10

pemphigus vegetans is associated with what disorder

Answer 10

ulcerative colitis

Question 11

what is seen in pemphigus vegetans

Answer 11

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

Question 12

pemphigus foliaceus has what target antigen

Answer 12

Dsg 1

Question 13

what is seen in pemphigus foliaceus

Answer 13

superficial, subcorneal blister

Question 14

what are the target antigens in bollous pemphigoid

Answer 14

BPAg1 and BPAg2 in the hemidesmosome

only BPAg2 is known to cause blisters

Question 15

bollus pemphigoid is typically found at what sites of what patients

Answer 15

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

Question 16

what is seen histologically in bollus pemphigoid

Answer 16

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

Question 17

acantholytic blisters are seen in what

Answer 17

pemphigus vulgaris

Question 18

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

Answer 18

bollus pemphigoid

Question 19

what type of lesion is seen in bollous pemphigoid

Answer 19

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

Question 20

dermatitis herpetiformis is seen in what patients

Answer 20

20-60 years old

2:1 M:F ratio

Question 21

dermatitis herpetiformis is due to what

Answer 21

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

Question 22

dermatitis herpetiformis is associated with what

Answer 22

HLA-B8
HLA-DR
HLA-DQ

Question 23

the lesions in dermatitis herpetiformis appear how

Answer 23

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

Question 24

dermatitis herpetiformis is associated with what disorder

Answer 24

Celiac disease

Question 25

dermatitis herpetiformis is typically found at what sites

Answer 25

buttocks, elbows, knees, scapular areas

Question 26

what is seen histologically in dermatitis herpetiformis

Answer 26

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

Question 27

how is bollous pemphigoid diagnosed

Answer 27

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

Question 28

epidermolysis bullosa is what

Answer 28

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

Question 29

what is common in all forms of epidermolysis bullosa

Answer 29

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

Question 30

epidermolysis bullosa simplex is caused by what

Answer 30

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

Question 31

junctional epidermolysis bullosa is characterized by what

Answer 31

blisters formed from separation of lamina lucida

Question 32

dystrophic epidermolysis bullosa is characterized by what

Answer 32

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

Question 33

non-herlitz junctional epidermolysis bulls is characterized by what

Answer 33

laminin V(beta)3 defect

Question 34

what is seen histologically in epidermolysis bullosa

Answer 34

``` 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 ```

Question 35

porphyria cutanea tarda is seen in what patients

Answer 35

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

Question 36

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

Answer 36

``` uroporphyrinogen decarboylase (UROD) excessive accumulation of porphyrins ```

Question 37

what is type I porphyria cutanea tarda

Answer 37

acquired disease via exposure to drugs/chemicals | hepatic UROD is inhibited

Question 38

what is Type II prophyria cutanea tarda

Answer 38

autosomal dominant | deficient in RBCs and fibroblasts

Question 39

what does the accumulation of porphyrins do in porphyria cutanea tarda

Answer 39

makes skin photosensitive

Question 40

what is seen in prophyria cutanea tarda

Answer 40

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

Question 41

what exacerbates porphyria cutanea tarda

Answer 41

``` iron alcohol smoking estrogens hepatitis C HIV halogenated hydrocarbons ```

Question 42

how is porphyria cutanea tarda diagnosed

Answer 42

test for uroporphyrin in urine | orange color under woods lamp

Question 43

what fluoresces orange under a woods lamp

Answer 43

porphyria cutanea tarda

Question 44

what histological features are seen in porphyria cutanea tarda

Answer 44

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