Dermis/Epiderms/BMZ

Question 1

What are two key antigens in the hemidesmisomes

Answer 1

BP230 = BP1
BP180 = BP2 = Col XVII

Question 2

protein in plankin family, in cytoplasm of BMX key for cytoskeletal

Answer 2

BP230 or BP1

Question 3

rereffed to type XVII col, transmambrane protein that connects basal keritinocytes via BP2 to the cytoskeleton AND connector between lamin332 and Col VII

Answer 3

BP180 or BP2

Question 4

most comon autoimmune bullous dermatosis; in elderly

Answer 4

Bullous pemphigoid

Question 5

Tense blisters, usually serous and very stable because whole roof is epiderms

Answer 5

Bullous Pemphigoid

Question 6

What antiB are directed against BMZ in bullous pemphigoid

Answer 6

Against BP 230 and 180 or BP1 and BP2 … remeber BR2 = Col XVII == subepidermal blisters

Question 7

What do we see on DIF for Bullous pemphigoid

What about IFF

Answer 7

DIF: linear IgG and C3 along BMX
IFF: linear stain on epidermal side

Question 8

Which has better prognosis: BP or pemphigus vulgaris?

Answer 8

BPf

Question 9

TX for bulluous P?

Answer 9

oral steroids and immunosuppresents for severe

Question 10

Mutated BP180 only

Answer 10

GABEB; see big blister on legs, bad teeth, bad hair

–Lack of BP180 in all the immunoflourscents

Question 11

immediately under hemidesmisomes, thin and only key compomenet is BP180

Answer 11

Laminia Lucida

Question 12

What is lamina densa made of

Answer 12

Typve IV and laminins
Key is that laminin 332 binds to hemidesmosomal integerin alpha4B4 on basal keritinocytes and to type VII collagen in dermis providing adhesions

Question 13

seen old people w/ recurrent blisters in mucous… see scars and strictures and blindness

Answer 13

Mucous membran pemphigoid

Question 14

Proteins wrong in MMP?

Answer 14

most have antiB against BP180 adn some have aB vs BP230 or integrin B4 and laminin332

Question 15

What form of MMP associated with malignancies?

Answer 15

antilammin 332

Question 16

What do we see in DIF and IFF in MMP

Answer 16

linear IgG and C3 deposits and see staining on the epidermal, dermal and dermal side (when 332 involved)

Question 17

What does sublamina Densa contains?

Answer 17

Type VII; 3 alpha chains that form anchoring fibrils
—type VII key to maintain epidermal-dermal cohesion bc binds to both Type and type IV collagens to join lamina densa and papillary densa

Question 18

rara autoimmune bullous dermatosis that with SLIGHT trauma get issues; see on hands, knees, elbows and healing leaves milia, scars and pigment disorders
often invovles oral mucosa

Answer 18

Epidermolysis Bullosa Aquisitia (EBA)

Question 19

What can we see in severe cases of EBA?

Answer 19

fibrosis of hands and ffet with nail dystrophy

Question 20

Target antiG in EBA?

Answer 20

Type VII collagen (key in sublimina densa)

Question 21

DIF and IFF for EBA

Answer 21

IgG and C3 along BMZ and see IIF on the DERMAL side

Question 22

Interited Epidermolysis Bullosa

1. EB simplex:
2. Junction EB
3. Dystrophic EB
4. Kindler syndrom

Answer 22

1. keratin 5, 14
2. laminin, BP180, integrins
3. type VII
4. Kindler