Desmoglein
Pemphigus Vulgaris
Pemphigus Foliaceous
Pemphigus Variants
Random
100
Pemphigus involves autoantibodies (IgA or IgG) directed at various proteins in what complex?
Desmosomes!
100
PV involes autantibodies to what?
Desmolgein 3 (mucosal dominant pemphigus) OR
Dsg1 and Dsg 3 (mucocutaneous pemphigus)
100
PF is the second most common for of pemphigus except in what locations?
Brazil
Tunisia
Finland
100
Presentation of IgA Pemphigus? (think morphology and most common locations)
flaccid vesicles/pustules in annular/circinate pattern
most common on axillae and groin
100
Path of PV?
Eosinophilic spongiosis (earliest finding) and then subrabasilar acantholysis with tombstoning and extensive hair follicle involvement
200
Where is desmoglein 1 expressed?
Expressed in ALL LEVELS of the epidermis (top > bottom)
200
Most common mucosal sites affected by PV?
Buccal and palatine mucosa
200
Name of endemic variant of PV that has highest incidence in rural Brazilian towns where rivers are rich in Black flies (simulium spp)
Fogo Selvagem
200
What are the two types of IgA pemphigus
Subcorneal pustular dermatosis type
Intraepidermal neutrophilic type
200
Path of Pemphigus Foliaceous?
subcorneal acantholysis (granular layer > midlevel epidermis)
*DIF same as PV but upper epidermis most intensely involved
300
Where is Desmoglein 3 expressed?
mostly in lower portion of epidermis and throughout mucosal epithelium
300
Pemphigus Vegetans is a subtype of PV. How is it different?
Affects intertriginous area > scalp and face
Early lesions are flaccid pustules rather than vesicles
300
Name of pemphigus foliaceous variant that has an overlap with lupus with localization of the malar region of the face and other seborrheic areas
Pemphigus Erythematosus (Senear-Usher Syndrome)
*DIF shows intercellular pemphigus pattern with granular to linear IgG and C3 along BMZ
300
Pemphigus variant that resembles DH?
Pemphigus Herpetiformis
300
Best IIF substrate for Paraneoplastic Pemphigus
Rat Bladder
400
Where does Desmoglein 4 have an important role?
in hair follicles
400
What does DIF of PV show?
chicken wire staining with IgG ± C3, with lower epidermis most strongly involved
400
Clinical presentation of PF?
impetigo like crusted erosions that favor a seborrheic distribution (face, scalp, central upper trunk). Lesions or plaques can have "cornflake" like scale
400
Target antigen in Subcorneal Pustular Dermatosis type of IgA Pemphigus?
Desmocollin 1
400
Autoantibodies targeted in paraneoplastic pemphigus?
Entire plakin family and Dsg 1 and Dsg3
500
In what two diseases is Desmoglein 4 mutated?
AR localized hypotrichosis
AR monilethrix
500
Best Indirect IF substrate for Pemphigus Vulgaris?
Monkey esophagus
500
PF involves autoantibodies to what?
Desmoglein 1
500
Most common causes of drug induced pemphigus?
Penicillamine and ACE inhibitors
500
Most common cancers associated with paraneoplastic pemphigus? (4)
NHL > CLL > Castleman Disease (#1 in children) > Thymoma