Flashcards in Dermis/Epiderms/BMZ Deck (22)
What are two key antigens in the hemidesmisomes
BP230 = BP1
BP180 = BP2 = Col XVII
protein in plankin family, in cytoplasm of BMX key for cytoskeletal
BP230 or BP1
rereffed to type XVII col, transmambrane protein that connects basal keritinocytes via BP2 to the cytoskeleton AND connector between lamin332 and Col VII
BP180 or BP2
most comon autoimmune bullous dermatosis; in elderly
Tense blisters, usually serous and very stable because whole roof is epiderms
What antiB are directed against BMZ in bullous pemphigoid
Against BP 230 and 180 or BP1 and BP2 ... remeber BR2 = Col XVII == subepidermal blisters
What do we see on DIF for Bullous pemphigoid
What about IFF
DIF: linear IgG and C3 along BMX
IFF: linear stain on epidermal side
Which has better prognosis: BP or pemphigus vulgaris?
TX for bulluous P?
oral steroids and immunosuppresents for severe
Mutated BP180 only
GABEB; see big blister on legs, bad teeth, bad hair
--Lack of BP180 in all the immunoflourscents
immediately under hemidesmisomes, thin and only key compomenet is BP180
What is lamina densa made of
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
seen old people w/ recurrent blisters in mucous... see scars and strictures and blindness
Mucous membran pemphigoid
Proteins wrong in MMP?
most have antiB against BP180 adn some have aB vs BP230 or integrin B4 and laminin332
What form of MMP associated with malignancies?
What do we see in DIF and IFF in MMP
linear IgG and C3 deposits and see staining on the epidermal, dermal and dermal side (when 332 involved)
What does sublamina Densa contains?
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
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
Epidermolysis Bullosa Aquisitia (EBA)
What can we see in severe cases of EBA?
fibrosis of hands and ffet with nail dystrophy
Target antiG in EBA?
Type VII collagen (key in sublimina densa)
DIF and IFF for EBA
IgG and C3 along BMZ and see IIF on the DERMAL side