53 - Paraneoplastic Pemphigus

Question 1

Malignancies associated with paraneoplastic pemphigus

Answer 1

Non-Hodgkin lymphoma
Chronic lymphocytic leukemia
Castleman disease

Question 2

In children with PNO, _____ is almost always the underlying neoplasm

Answer 2

Castleman disease

Question 3

Patients with PNP have evidence of markedly elevated levels of IL-

Answer 3

6

Question 4

Pathogenic autoantibodies from PNP patients bind to the middle portion of desmoglein 3 — extracellular domains _____ — in contrast to PV patients where the pathogenic autoantibodies bond to extracellular domain _____

Answer 4

2 and 3

1

Question 5

Dominant response in the lichenoid variant of PNP

Answer 5

Interface dermatitis caused by desmoglein 3-specific CD4+ cells

Question 6

Ectopic desmoglein 3 expression is present in the

Answer 6

Lungs

Question 7

Probably the cause for the fatal bronchiolitis obliterans involvement in PNP

Answer 7

Squamous metaplasia of the lungs

Question 8

Most constant clinical feature of PNP

Answer 8

Intractable stomatitis

Question 9

Earliest presenting sign and the one feature that persists throughout the course of PNP

Answer 9

Intractable stomatitis

Question 10

Oral lesions in PNP preferentially localize to the _____ and characteristically extend onto and involve the

Answer 10

Lateral borders of the tongue

Vermilion of the lips

Question 11

Helps distinguish PNP from PV

Answer 11

PNP - common presence of both blisters and lichenoid lesions affecting the palms and soles as well as the paronychial tissues
PV - acral and paronychial lesions are uncommon

Question 12

PNP is the only form of pemphigus that involves

Answer 12

Non-stratified squamous epithelium

Question 13

Key finding jn PNP is the serologic identification of polyclonal IgG autoantibodies against _____ and in most cases _____

Answer 13

Plakin proteins

Desmogleins 1 and 3

Question 14

Autoantibodies against these proteins are the most characteristic surrogate markers for PNP

Answer 14

Plakin - envoplakin and periplakin

Question 15

To screen for PNP autoantibodies, one can test for IgG autoantibodies by IIF reactivate with _____ epithelium

Answer 15

Rodent urinary bladder

Question 16

Patients develop autoantibodies against structurally related plakin proteins and structurally unrelated transmembrane cell-surface proteins (desmogleins) that are physically linked to the plakin proteins in the desmosome and hemidesmosome

Answer 16

Epitope spreading

Question 17

Most sensitive and specific test for demonstration of antiplakin antibodies in PNP

Answer 17

Immunoprecipitation

Question 18

Histopathology of PNP vs PV and PF

Answer 18

PNP - variability in histologic findings; findings result from cell-mediated cytotoxicity

Question 19

Biopsy findings of noninflammatory cutaneous blisters

Answer 19

Suprabasilar acantholysis

Question 20

Biopsy findings of erythematous macules and papules

Answer 20

Interface and lichenoid dermatitis

Question 21

Most effective and efficient method for screening for occult tumors

Answer 21

CT or MRI of the body from the neck to the base of the bladder

Question 22

Treatment for individuals with benign or encapsulated tumors

Answer 22

Surgical excision

Question 23

Treatment for patients with malignant neoplasms

Answer 23

No consensus regarding a therapeutic regiment that is consistently effective