Chapter 30 - Pemphigus Flashcards Preview

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Flashcards in Chapter 30 - Pemphigus Deck (24):
1

What are the 3 major forms of pemphigus?

1) Pemphigus vulgaris, 2) pemphigus foliaceus, 3) paraneoplastic pemphigus. The other forms include 4) drug induced, 5) IgA pemphigus

2

True/False: All pemphigus foliaceus patients will have mucosal membrane erosions.

False. Pemphigus foliaceus patients only have cutaenous involvement without mucosal lesions.

3

What are the 2 major subgroups of cadherins?

Classic and desmosomal, which include desmogleins and desmocollins

4

What are differences between adherens junctions and desmosomes? Hint: what do they anchor, which cadherins do they contain, what are the cytoplasmic components

1) Adherens junctions-anchor actin microfilament bundles, contain classic cadherins, alpha & beta catenin, plakoglobin (quick but weak cellular adhesions); 2) desmosomes-anchor intermediate filaments, contain desmosomal cadherins, plakoglobin, plakophilin, desmoplakin (slow but strong cellular adhesions)

5

What are the 4 isoforms of desmogleins and where are they found?

Dsg1&3-stratified squamous epithelia; Dsg2-all desmosomes including simple epithelia, myocardium; Dsg4-hair follicles

6

Which Ag is cleaved by exfoliative toxins such as those produced by S. aureus?

Dsg1. Leads to bullous impetigo, SSSS

7

What mucosal membrane sites can be involved in PV?

Oral cavity, esophagus, conjunctivae, nasal mucosa, vagina, penis, anus, labia

8

True/False: PV lesions tend not to heal and those that do, heal with hyperpigmentation and scarring.

False, they heal with NO scarring

9

Which drugs are most commonly associated with drug induced pemphigus?

Penicillamine, captopril. Most patients go into remission with discontinuation

10

Which neoplasms are commonly associated with paraneoplastic pemphigus?

Non-Hodgkin lymphoma, chronic lymphocytic leukemia, Castleman's disease, thymomas, sarcomas, Waldenstrom's macroglobulinemia

11

What is the most constant clinical feature of paraneoplastic pemphigus?

Stomatitis, which often extend onto vermilion lip. Severe, presents early on, persists and extremely resistant to treatment

12

What test is the most reliable and sensitive diagnostic test for pemphigus?

Direct IF (IgG deposition) on skin bx

13

What is the standard treatment for PV?

Prednisone. Clinical improvement is based on number of new blisters/day, rate of healing. Response in 3-7d

14

What is the drug of choice for IgA pemphigus?

Dapsone. Response in 1-2d

15

What is a good treatment option in localized pemphigus foliaceus?

Potent topical steroids

16

True or false: there are no good treatment guidelines for paraneoplastic pemphigus.

True

17

True or false: continuous cardiac monitoring is necessary with methylprednisone IV administration.

True; this is due to the potential for electrolyte shifts and resultant cardiac arrest

18

True or false: ELISA can distinguish between pemphigus vulgaris that involves only Dsg3 and pemphigus vulgaris that involves both Dsg 1 and Dsg3.

True

19

What type of animal substrate is typically used for indirect IF to detect paraneoplastic pemphigus?

Rat bladder, because it contains transitional epithelium, which will bind antiplakin antibodies

20

What subclass of IgG is most commonly implicated in pemphigus?

IgG4, which does not fix complement

21

True or false: DIF is the most reliable and sensitive diagnostic test for all forms of pemphigus.

True

22

What is the eponymous name for pemphigus erythematosus?

Senear-Usher syndrome

23

True or false: pruritus is often a common symptom associated with IgA pemphigus.

True

24

Roughly what percentage of patients with paraneoplastic pemphigus are already aware of their underlying malignancy?

2/3 (66%)