Immunobullous Flashcards
Target for pemphigus vulgaris
Dsg3, Dsg1
Target for pemphigus foliaceus
Dsg1
Target for Paraneoplastic pemphigus
Dsg1, 3, Desmoplakin Envoplakin Periplakin Plectin BPAG1
Target for IgA pemphigus
Demoscollin 1
Target for Pemphigus Vegetans
Dsg 3+/- 1
Target for pemphigus erythematous
Dsg1
Target for bullous pemphigoid
NC16A domain of BPAG1 and BPAG2
Target for Herpes gestationis
NC16A domain of BPAG2 and BPAG1
Target for cicatricial pemphigoid
C-terminal of BPAG2, Laminin 5, ocular subtype is beta-4-integrin
Target for epidermolysis bullosa acquisita
Collagen VII
Target for Linear IgA disease
LAD1 antigen (BPAG2; 97kDa portion)
Target for Bullous SLE
Collagen VII
T/F - pemphigus has an average disease duration of 10 y
True
rituximab infusion protocol
1g infusion, Week 0, Week 2 then repeat dose at 6-12 mo
histo and DIF of pemphigus foliaceus
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
T/F pemphigus foliaceus has a positive Nikolsky sign
T
T/F in pemphigus vegetans, oral mucosal involvement is almost invariable often with cerebriform changes to the tongue
T
List Ddx of pemphigus vegetans
- Hailey Hailey, pemphigoid vegetans (especially in major body folds), Blastomycosis like pyoderma, pyodermatitis, pyostomatitis vegetans
What is pemphigus herpetiformis
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
What is Senear-Usher (pemphigus erythematosus)
type of pemphigus FOLIACEUS. Dsg 1 is target. Pt have features of LE and pemphigus.
T/F Progression to SLE is common with Senear Usher
Rare
T/F Pemphigus erythematosus can simulate LE or Seb term with erythematous rash n a butterfly distribution
T
2 types of IgA pemphigus
1 - intraepidermal neutrophilic
2 - sub corneal pustular dermatosis
–> both are clinically indistinguishable from sub corneal pustular dermatosis
IgA pemphigus commonly has mucosal involvement
F unusual
IgA pemphigus associated with monoclonal IgA gammopathy
T
Ddx of IgA pemphigus
Subcorneal pustular dermatosis, pemphigus foliaceus, bullous impetigo, dermatitis herpetiformis, linear IgA, pustular psoriasis
Tumours associated with paraneoplastic pemphigus and their frequency
NHL 44% CLL 19% Castleman's Thymoma Retroperitoneal sarcoma Waldenstrom's macroglobulinaemia 4%
extracutaneous manifestations of pemphigus vulgaris
obstructive lung disease, bronchiolitis obliterates, death `
drug triggers of BP
Thiazides Infliximab NSAIDs Duiretics, DPP4 inh ACE inh
natural hx of BP
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`
targets in mucous membrane pemphigoid
BP230, BP180, Type XVII (17) collagen, laminin 332, B4 subunit of a6b4 integrin
T/F get binding on both roof and floor for MMP
T
T/F indirect antibodies seen in majority of MMP
F 20-30%
common presentation of EBA
- non inflammatory mechanobullous disease
- -> Acral blisters that heal with atrophic scarring, milia formation and dyspigmentation
- ->cutaneous blisters become haemorrhagic
T/F EBA can lead to mitten deformity
T
what % of EBA have scalp involvement
20%
associations of EBA
IBD + cutaneous Crohn’s, MM , LE, RA , thyroiditis, DM
inflammatory EBA can be indistinguishable from BP, MMP, Brunsting-Perryvariant of MMP
T
what % of pt with linear IgA have mucosal involvement
70%
time to remission for dermatitis herpetiformis
3-4 mo of gluten free therapy needs for a response, though concurrent suppression of symptoms with dapsone is usually necessary
which drugs induce linear iga
Victoria Loves FACD Vanc Lithium Frusemide atorvastatin captopril diclofenac
