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Derm 2020 > Immunobullous > Flashcards

Immunobullous Flashcards

1
Q

Target for pemphigus vulgaris

A

Dsg3, Dsg1

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2
Q

Target for pemphigus foliaceus

A

Dsg1

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3
Q

Target for Paraneoplastic pemphigus

A 
Dsg1, 3,
Desmoplakin
Envoplakin
Periplakin
Plectin
BPAG1
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4
Q

Target for IgA pemphigus

A

Demoscollin 1

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5
Q

Target for Pemphigus Vegetans

A

Dsg 3+/- 1

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6
Q

Target for pemphigus erythematous

A

Dsg1

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7
Q

Target for bullous pemphigoid

A

NC16A domain of BPAG1 and BPAG2

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8
Q

Target for Herpes gestationis

A

NC16A domain of BPAG2 and BPAG1

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9
Q

Target for cicatricial pemphigoid

A

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

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10
Q

Target for epidermolysis bullosa acquisita

A

Collagen VII

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11
Q

Target for Linear IgA disease

A

LAD1 antigen (BPAG2; 97kDa portion)

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12
Q

Target for Bullous SLE

A

Collagen VII

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13
Q

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

A

True

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14
Q

rituximab infusion protocol

A

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

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15
Q

histo and DIF of pemphigus foliaceus

A

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

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16
Q

T/F pemphigus foliaceus has a positive Nikolsky sign

A

T

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17
Q

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

18
Q

List Ddx of pemphigus vegetans

A
  • Hailey Hailey, pemphigoid vegetans (especially in major body folds), Blastomycosis like pyoderma, pyodermatitis, pyostomatitis vegetans
19
Q

What is pemphigus herpetiformis

A

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

20
Q

What is Senear-Usher (pemphigus erythematosus)

A

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

21
Q

T/F Progression to SLE is common with Senear Usher

22
Q

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

23
Q

2 types of IgA pemphigus

A

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

24
Q

IgA pemphigus commonly has mucosal involvement

25
Q

IgA pemphigus associated with monoclonal IgA gammopathy

26
Q

Ddx of IgA pemphigus

A

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

27
Q

Tumours associated with paraneoplastic pemphigus and their frequency

A 
NHL 44%
CLL 19%
Castleman's
Thymoma
Retroperitoneal sarcoma
Waldenstrom's macroglobulinaemia 4%
28
Q

extracutaneous manifestations of pemphigus vulgaris

A

obstructive lung disease, bronchiolitis obliterates, death `

29
Q

drug triggers of BP

A 
Thiazides
Infliximab
NSAIDs
Duiretics, DPP4 inh 
ACE inh
30
Q

natural hx of BP

A

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`
31
Q

targets in mucous membrane pemphigoid

A

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

32
Q

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

33
Q

T/F indirect antibodies seen in majority of MMP

34
Q

common presentation of EBA

A
  • non inflammatory mechanobullous disease
  • -> Acral blisters that heal with atrophic scarring, milia formation and dyspigmentation
  • ->cutaneous blisters become haemorrhagic
35
Q

T/F EBA can lead to mitten deformity

36
Q

what % of EBA have scalp involvement

37
Q

associations of EBA

A

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

38
Q

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

39
Q

what % of pt with linear IgA have mucosal involvement

40
Q

time to remission for dermatitis herpetiformis

A

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

41
Q

which drugs induce linear iga

A 
Victoria Loves FACD
Vanc
Lithium
Frusemide
atorvastatin
captopril
diclofenac

Derm 2020 (59 decks)

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