9 - 53 - PARANEOPLASTIC PEMPHIGUS

Question 1

Which is/are associated with a poor prognosis in BP?

a. Extensive disease

b. anti-BP180 antibodies

c. Old age

d. all of the above

Answer 1

B. p.952

Question 2

What is the mainstay of therapy of BP?

a. Topical steroids

b. Oral steroids

c. Methotrexate

d. Rituximab

Answer 2

B

Question 3

1 Which of the following is false regarding defining features of paraneoplastic pemphigus?

A. Painful stomatitis and a monomorphous cutaneous eruption

B. Histologic fifindings that reflflect cutaneous lesions (acantholysis, lichenoid or interface changes)

C. IgG complement deposition in the epidermal intercellular spaces and/or along basement membrane zone with granular/linear component

D. Serum IgG autoantibodies that bind of cell surface of skin and mucosae, but also to simple, columnar and transitional epithelia

Answer 3

A

Question 4

In paraneoplastic pemphigus, the most common associated neoplasm in adults is _______ .

A. Castleman disease

B. Chronic lymphocytic leukemia

C. Non Hodgkin lymphoma

D. Thymoma

Answer 4

C

Question 5

In paraneoplastic pemphigus, the most common associated neoplasm in children is _______ .

A. Castleman disease

B. Chronic lymphocytic leukemia

C. Non Hodgkin lymphoma

D. Thymoma

Answer 5

A

Question 6

The most constant clinical feature of paraneoplastic pemphigus is the presence of _______ .

A. Cutaneous blisters

B. Intractable stomatitis

C. Dysphagia

D. Dyspareunia

Answer 6

B

Question 6

The key finding in paraneoplastic pemphigus is the serologic identification of polyclonal IgG autoantibodies against _______ .

A. desmogleins 1 and 3

B. plakin proteins

C. a 2 - macroglobulin-like-1

D. BP180

Answer 6

B

Question 7

neoplasms most commonly associated with PNP

Answer 7

non-Hodgkin lymphoma, chronic lymphocytic leukemia, or Castleman disease

Other:
- thymoma
- sarcomas
- waldenstrom macroglobulinemia
- myasthenia gravis

Question 8

In PNP, autoantibodies are directed at what proteins?

Answer 8

desmogleins 1 and 3, desmoplakins, envoplakin and periplakin

Question 9

Substrate used for IIF in testing PNP

Answer 9

rodent bladder epithelium

Question 10

what are the defining features of PNP?

Answer 10

Question 11

most characteristic and consistently recognized plakin antigens in PNP

Answer 11

Envoplakin and Periplakin

Question 12

the only form of pemphigus that involves non-stratified epithelium

Answer 12

Paraneoplastic Pemphigus

Question 13

studies performed on 2 different series of PNP patients revealed a significant predominance of what HLA-classes?

Answer 13

HLA-class II DRB∗03 and HLA-class I Cw∗14 genes

Question 14

Patients with PNP have evidence of markedly elevated levels of what interleukin?

Answer 14

IL-6

Question 15

most constant clinical feature of PNP

Answer 15

Intractable stomatitis

Question 16

the earliest presenting sign and the one feature that persists throughout the course of the disease

Answer 16

Intractable stomatitis

Question 17

how do the mucosal lesions of PNP differ from that of PV?

Answer 17

PNP mucosal lesions show MORE NECROSIS AND LICHENOID CHANGE

Question 18

area of predlection of mucosal lesions of PNP?

Answer 18

lateral borders of the tongue, and characteristically extend onto and involve the vermilion of the lips

Question 19

blisters of PNP initially involve what areas?

Answer 19

upper trunk, head and neck, and proximal extremities

Question 20

how can you differentiate erythema multiforme and epidermal necrolysis vs PNP based of clinical course?

Answer 20

erythema multiforme and TEN are self-limited events that evolve and resolve over several weeks, whereas PNP is a relentlessly progressive and evolves continuously over months

Question 21

how can you differentiate PNP and PV based on cutaneous involvement of palms, soles and paronychial tissue?

Answer 21

The common presence of both blisters and lichenoid lesions affecting the palms and the soles as well as the paronychial tissues helps distinguish PNP from pemphigus vulgaris, in which acral and paronychial lesions are uncommon.

Question 22

severe pulmonary complication of PNP?

Answer 22

bronchiolitis obliterans

Question 23

What does it mean when IgG autoantibodies by indirect immunofluorescence are reactive with rodent urinary bladder epithelium?

Answer 23

A positive result implies the presence of plakin autoantibodies; however, the sensitivity and specificity of this serologic test are only approximately 75% and 83%, respectively

Question 24

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

Answer 24

Immunoprecipitation using radiolabeled, nondenatured epidermal extracts and serum from a patient with PNP and PV

Question 25

Target Antigens in Paraneoplastic Pemphigus

Answer 25

Question 26

Typical Histologic Findings of PNP

Answer 26

Question 27

In noninflammatory cutaneous blisters, what histopath finding is expected to be more prominent?

Answer 27

suprabasilar acantholysis

Question 28

When erythematous macules and papules are sampled, what histopath finding is predominant?

Answer 28

interface and lichenoid dermatitis

Question 29

DIF finding of PNP

Answer 29

deposition of immunoglobulin G and complement components on both the surface of basilar and suprabasilar keratinocytes and along the epidermal basement membrane zone

or

Immunoglobulin G and C3 on cell surfaces and along the basement membrane (false negatives common)

Question 30

Question 31

common terminal event

Answer 31

Respiratory failure

The development of shortness of breath with obstructive disease progressing to bronchiolitis obliterans is a terminal complication in most cases. Because these patients have autoantibodies that react with desmoplakins, and because desmoplakins are present in respiratory epithelium, respiratory failure may be a result of autoantibody-mediated injury to bronchial epithelium, with plugging of terminal bronchioles, resulting in airflow obstruction and ventilation–perfusion abnormalities.
Additionally, direct damage to alveolar epithelium could cause a diffusion barrier and subsequent intractable hypoxia.

Question 32

T/F. Treatment of the primary malignancy does not affect the activity of the autoimmune disease.

Answer 32

True

Question 33

First-line treatment options for PNP

Answer 33