BMZ

Question 1

immunobullous diseases

Answer 1

vesicles, bullae, erosions

inflam. directed against BMZ, mucosa may be affected

**antibodies

Question 2

mechanobullous diseases

Answer 2

absence of key structures that causes skin fragility, loss of anchoring function

Question 3

BMZ parts

Answer 3

1. plasma mem and hemidesmosomal plaque of basal keratinocyte
2. more superficial lamina lucida: traversed by BPAG2, clear band
3. underlying lamina densa: laminins and coll IV
4. sublamina densa: collagen (VII) and elastin

Question 4

anchoring proteins in hemidesmo

Answer 4

BPAG1: intracyto, binds to keratin intermed. filaments

BPAG2: transmem, type XVII collagen, binds BPAG1 and integrins in hemidesmo w/ laminin 5 in lamina densa

integrins

Question 5

laminin 5

Answer 5

key laminin in BMZ linking BPAG2, lamina densa, and anchoring fibrils of sublamina densa

Question 6

collagen IV

Answer 6

2 identical alpha helical subunits and 1 dissimilar alpha helical subunit –> triple helix –> tetrad –> lattice

Question 7

collagen VII

Answer 7

3 identical alpha chains –> anchoring fibrils

only in BM of st. sq. epi,

term. end insert into lamina dens –> loop w/ coll I and III

Question 8

coll III

Answer 8

more in fetal skin and wound healing

Question 9

vesicles vs. bullae

Answer 9

vesicle= blister .5cm

Question 10

pemphigus vulgaris (PV)

Answer 10

autoimmune blistering disease flaccid bullae
+ Nikolsky
oral erosions
scalp crusting

antibodies against desmo 3, but basal keratinocytes still connected to BMZ –> tombstoning

immunosuppressants, systemic steriods

Question 11

bullous pemphigoid (BP)

Answer 11

elderly
tense bullae on trunk and extrem.
- Nikolsky
+/- itching, - pain, - oral

antibodies against BPAG1 and BPAG2 –> spilt at level of hemidesmo
linear staining of BM w/ IgG and C3

topical steriods, oral anitbiotics (oral steriods and immunosupp for severe)

Question 12

cicatricial pemphigoid (CP)

Answer 12

aggressive, immunobullous, often chronic
favors mucosal surfaces
cutaneous blisters and erosions common on scalp, face, neck, upper trunk

antibodies against BPAG2 and laminin 5
biopsy–> subepiderm blister
linear IgG at lamina lucida

immunosupp (topical corticosteroids for mild)

Question 13

epidermolysis bullosa (EB)

Answer 13

mechobull.
bullae, blisters, erosions, and mechanical fragility

simple: auto dom, epiderm keratins absent
junctional: auto rec, laminin and BPAG2 mut in lamina densa
dystrophic: auto dom and rec, coll VII absent/abnorm

Question 14

dermatitis herpetiformis (DH)

Answer 14

vesiculobull disease not related to BMZ

N Euro descent
intense pruritic (occipital scalp, extensors, butt)
papules, vesicles, urticarial plaques
assoc w/ celiac disease and thyr disease

subepiderm blister w/ neutrophils
granular deposits of IgA in dermal papillae
anti-endomysial antibodies (anti-tissue transglutaminaase IgA)

gluten free diet, dapsone relieves itching