Immunobullous

Question 1

Target for pemphigus vulgaris

Answer 1

Dsg3, Dsg1

Question 2

Target for pemphigus foliaceus

Answer 2

Dsg1

Question 3

Target for Paraneoplastic pemphigus

Answer 3

Dsg1, 3,
Desmoplakin
Envoplakin
Periplakin
Plectin
BPAG1

Question 4

Target for IgA pemphigus

Answer 4

Demoscollin 1

Question 5

Target for Pemphigus Vegetans

Answer 5

Dsg 3+/- 1

Question 6

Target for pemphigus erythematous

Answer 6

Dsg1

Question 7

Target for bullous pemphigoid

Answer 7

NC16A domain of BPAG1 and BPAG2

Question 8

Target for Herpes gestationis

Answer 8

NC16A domain of BPAG2 and BPAG1

Question 9

Target for cicatricial pemphigoid

Answer 9

C-terminal of BPAG2, Laminin 5, ocular subtype is beta-4-integrin

Question 10

Target for epidermolysis bullosa acquisita

Answer 10

Collagen VII

Question 11

Target for Linear IgA disease

Answer 11

LAD1 antigen (BPAG2; 97kDa portion)

Question 12

Target for Bullous SLE

Answer 12

Collagen VII

Question 13

T/F - pemphigus has an average disease duration of 10 y

Answer 13

True

Question 14

rituximab infusion protocol

Answer 14

1g infusion, Week 0, Week 2 then repeat dose at 6-12 mo

Question 15

histo and DIF of pemphigus foliaceus

Answer 15

sub corneal vesicles and split with pacantholysis in the granular layer. perivascular lymphocytes and eosinophils. direct DIF has a netlike pattern with IgG and C3, which is more intense in the superficial dermis

Question 16

T/F pemphigus foliaceus has a positive Nikolsky sign

Answer 16

T

Question 17

T/F in pemphigus vegetans, oral mucosal involvement is almost invariable often with cerebriform changes to the tongue

Answer 17

T

Question 18

List Ddx of pemphigus vegetans

Answer 18

• Hailey Hailey, pemphigoid vegetans (especially in major body folds), Blastomycosis like pyoderma, pyodermatitis, pyostomatitis vegetans

Question 19

What is pemphigus herpetiformis

Answer 19

Clinically resembles DH. rare and atypical variant of pemphigus with target Dsg1. Characterised by 1. erythematous urticarial plaques with tense vesicles in a herpetiform arrangement; 2. eosinophilic spongiosis and sub corneal pustules with minimal or no apparent pacantholysis histologically; 3. IgG autoantibodies directed against the cell surfaces of keratinocytes

Question 20

What is Senear-Usher (pemphigus erythematosus)

Answer 20

type of pemphigus FOLIACEUS. Dsg 1 is target. Pt have features of LE and pemphigus.

Question 21

T/F Progression to SLE is common with Senear Usher

Answer 21

Rare

Question 22

T/F Pemphigus erythematosus can simulate LE or Seb term with erythematous rash n a butterfly distribution

Answer 22

T

Question 23

2 types of IgA pemphigus

Answer 23

1 - intraepidermal neutrophilic
2 - sub corneal pustular dermatosis
–> both are clinically indistinguishable from sub corneal pustular dermatosis

Question 24

IgA pemphigus commonly has mucosal involvement

Answer 24

F unusual

Question 25

IgA pemphigus associated with monoclonal IgA gammopathy

Answer 25

T

Question 26

Ddx of IgA pemphigus

Answer 26

Subcorneal pustular dermatosis, pemphigus foliaceus, bullous impetigo, dermatitis herpetiformis, linear IgA, pustular psoriasis

Question 27

Tumours associated with paraneoplastic pemphigus and their frequency

Answer 27

``` NHL 44% CLL 19% Castleman's Thymoma Retroperitoneal sarcoma Waldenstrom's macroglobulinaemia 4% ```

Question 28

extracutaneous manifestations of pemphigus vulgaris

Answer 28

obstructive lung disease, bronchiolitis obliterates, death `

Question 29

drug triggers of BP

Answer 29

``` Thiazides Infliximab NSAIDs Duiretics, DPP4 inh ACE inh ```

Question 30

natural hx of BP

Answer 30

30% relapse in first year of treatment, with extensive disease and associated dementia - risk factors for relapse --> demential and extensive disease - after cessation of therapy, 50% experience relapse in first 3 years`

Question 31

targets in mucous membrane pemphigoid

Answer 31

BP230, BP180, Type XVII (17) collagen, laminin 332, B4 subunit of a6b4 integrin

Question 32

T/F get binding on both roof and floor for MMP

Answer 32

T

Question 33

T/F indirect antibodies seen in majority of MMP

Answer 33

F 20-30%

Question 34

common presentation of EBA

Answer 34

- non inflammatory mechanobullous disease - -> Acral blisters that heal with atrophic scarring, milia formation and dyspigmentation - ->cutaneous blisters become haemorrhagic

Question 35

T/F EBA can lead to mitten deformity

Answer 35

T

Question 36

what % of EBA have scalp involvement

Answer 36

20%

Question 37

associations of EBA

Answer 37

IBD + cutaneous Crohn's, MM , LE, RA , thyroiditis, DM

Question 38

inflammatory EBA can be indistinguishable from BP, MMP, Brunsting-Perryvariant of MMP

Answer 38

T

Question 39

what % of pt with linear IgA have mucosal involvement

Answer 39

70%

Question 40

time to remission for dermatitis herpetiformis

Answer 40

3-4 mo of gluten free therapy needs for a response, though concurrent suppression of symptoms with dapsone is usually necessary

Question 41

which drugs induce linear iga

Answer 41

``` Victoria Loves FACD Vanc Lithium Frusemide atorvastatin captopril diclofenac ```