Vesiculobullous Disease Basic Science 1 Flashcards
Name the cadherins.
Calcium dependent
Classic-E, P, & N-cadherin
Desmosomal-Desmoglein, Desmocollin
Name the armadillo family proteins
Plakoglobin, plakophilin, β-catenin
Name the plakins and what is there function?
adhesion of keratin intermediate filaments
Desmoplakin, BPAg1, plectin, envoplakin, periplakin
The lamina densa is composed mainly of what type of collagen?
Type IV
The anchoring fibrils contain what type of collagen?
Type VII-Bullous SLE, EBA, DEB
What creates the negative charge in the lamina densa?
Perlecan-heparin sulfate progeoglycan
What tissue do you use for indirect immunoflourescence for pemphigus vulgaris? paraneoplastic pemphigus? Pemphigus foliaceus?
PV-Monkey’s are vulgar, PNP-Rat’s I have cancer, PF-Guinea pigs eat foliage
What is Nikolski’s sign?
slight pressure, twisting, or rubbing on skin shears epidermis, reveals dermis
What is Asboe-Hansen’s sign?
“bulla spreading phenomenon” blisters spread laterally w/ pressure on surface of blister
What is the antibody present in the mucosal variant of pemphigus vulgaris?
Desmoglein 3 (130 kD)
What antibody is present in mucocutatneous pemphigus vulgaris?
Desmoglein 1 (160kD) & 3 (130kD)
What HLA is the most common for pemphigus vulgaris?
DR4, DR6
What is the standard histopathology for pemphigus vulgaris?
Suprabasilar acantholysis with tombstoning involving hair follicle and sebaceous glands (differentiates from Hailey-Hailey)
What antibody is present in pemphigus foliaceus?
Desmoglein 1 (160kD)
Pemphigus foliaceus is more common than PV in what countries?
Finland, Tunisia, Brazil (everywhere else PV is much more common than PF)
What is the histology of pemphigus foliaceus?
Subcorneal /intragranular acantholysis with “cling ons”
What arthropod causes Fogo Sevalgem (endemic PF)?
Simulium nigrimanum-also causes onchocerciasis
What antibody do you have in pemphigus erythematosus?
anti desmoglein 1
What antibodies are present in paraneoplastic pemphigus?
everything but BPAg2
What is the most common malignancy associated with paraneoplastic pemphigus?
non-Hodgkins lymphoma (40%), CLL (30%), Castleman’s (10%-most common in children), Thymoma, Sarcoma & Waldenstrom’s macroglobulinemia (all 6%)
Ddx for subepidermal blister + neutrophils predominantly?
Herpetic LIPS Herpetic-DH L = lupus (bullous) I = linear IgA P = pemphigoid (BP) S = Sweet's syndrome
What is the DIF pattern in pemphigus vulgaris?
Chicken wire with IgG4
What are the two subtypes of pemphigus vegetans?
Neuman (MC and severe), and Hallopeau (mild, pustular)
Name the drugs implicated in pemphigus foliaceus?
Same as PV-Thiol drugs (captopril, penicillamine, gold thiosulfate), sulfa-releasing, penacillamine, nifedipine
