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Blistering disorders Melissa > Autoimmune blistering > Flashcards

Autoimmune blistering Flashcards

1
Q

Associations of EBA

A

IBD (esp crohns), myeloma, SLE, RA, thyroiditis, dermatomyositis, amyloidosis, lymphoma, carcinoma, drug induced.

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2
Q

Types of EBA

A

1) classic - acral with alopecia and nail dystrophy
2) BP like (no milia or scarring): widespread with accentuation in skin folds
3) mucous membrane including bursting-perry with scarring alopecia. oesophageal strictures, laryngeal stenosis, ocular scarring, blindness.

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3
Q

DIF of EBA

SSS

A

Continuous, linear, IgG at BMZ, can have C3, IgA or M

SSS: dermal side.

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4
Q

EBA can occur at any age

A

T

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5
Q

EBA is more common in asians

A

T

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6
Q

Antibodies in EBA

A 
alpha-chain 290kDa
NC1 domain (collagen VII) 145kDa
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7
Q

Where is the blister in EBA

A

sublamina densa

PMN predominant

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8
Q

BP antigens

A

BPAg1 230kDa

BPag2 NC16A 180kDa

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9
Q

BP IF

A

Linear C3 and IgG at BMZ

IgG4>G1

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10
Q

Drugs associated with BP

A

Fluid Sores Caused by Prescription

Frusemide, Spironolactone/sulphasalazine, Captopril, Betablockers, PCN, penicillamine.

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11
Q

Potential associations with BP

A

Psoriasis, LP, parkinsons, dementia, stroke, MS

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12
Q

Triggers for BP

A

trauma, burns, radiotherapy, UV irradiation

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13
Q

BP oral cavity in %

A

30%

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14
Q

Eosinophilia in BP %

A

50%

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15
Q

Gender predominance in BP

A

M>F

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16
Q

BP in children - where

A

acral and vulvar

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17
Q

EM in BP where is the blister

A

Lamina lucida blister

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18
Q

Variants of BP

A

Localised variants: pretibial, vulva, peristomal, umbilical, distal end of amputated limb, sites of radiotherapy, burns
Unusual: palmo/plantar, vegetans, nodularis, vesicular TEN like, papular, eczematous, erythrodermic, LP pemphigoides

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19
Q

Drugs associated with MMP

A

penicillamine, clonidine

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20
Q

Antibodies and MMP

A

Group 1 = laminin332=5, laminin 6
Group 2= just ocular: integrin beta4
Group 3: mucosa + skin BPAG2/BP/collagen XVII/BP230
Group 4: heterogenous. variable mucosa, no skin

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21
Q

% of oral mucosa in MMP

A

85%

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22
Q

skin involvement in MMP

A

25-30%

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23
Q

Associations with MMP

A

Anti-laminin 5 (332) and adenocarcinoma,

autoimmune disease: LS

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24
Q

DIF MMP

A

linear deposits of immunoglobulins and/or complement along epithelial BMZ. Linear IgG and C3 at BMZ
Mucosa 50-90% > skin 20-50%

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25
Q

SSS MMP

A

epidermal roof mostly

but anti-laminin 5 (332): dermal

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26
Q

histo MMP

A

blister in lamina lucida with mixed infiltrate: may have plasma cells, Eos, PMNs

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27
Q

Ddx of cicatricial pemphigoid

A

PV, erosive LP, ocular pseudopemphigoid, SJS/TEN, BP, EBA, linear IgA

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28
Q

Herpes gestationis DIF

A

linear C3 at BMZ

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29
Q

Associations with pemphigus gestationis

A

Graves, hydatidiform mole, choriocarcinoma.

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30
Q

Pemphigus gestationis antigens

A

BPAg2 NC16A

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31
Q

Pemphigus gestationis DIF

A

linear C3 at BMZ

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32
Q

Pemphigus gestationis - mucosal involvement?

A

No

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33
Q

Pemphigus gestationis - when

A

4 weeks to 5 weeks post part

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34
Q

Pemphigus gestationis who

A

father more often HLA-DR2

50% 1st pregnancy, 8% of subsequent pregnancies are spared.

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35
Q

Pemphigus gestationis flares

A

post partum, menstruation, OCP

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36
Q

Pemphigus gestationis more likely to be prolonged if:

A

older age, multiparity, mucosal involvement

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37
Q

Pemphigus gestationis fetal prognosis

A

worse when early onset. Risk of low birth weight and premature
neonatal PG in 3%

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38
Q

SSS MMP

A

Epidermal (roof) except anti-laminin 5 (332): floor

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39
Q

MMP antigens

A

BPAg2 - NC16A, BPAg1, Laminin 5, type VII collagen, alpha 6integrin beta 4

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40
Q

MMP M>F

A

F

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41
Q

DIF MMP

A

Linear IgG +/- complement along epithelial BMZ (mucosa >skin). Less commonly IgA and IgM

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42
Q

%+ve IIF MMP

A

20-30% usually IgG +/-IgA

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43
Q

What gives a worse prognosis in children with MMP

A

IgA

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44
Q

Associations with MMP

A

LS
antiliminin 5 - adenoca
Drugs: penicillamine, clonidine, NSAIDS

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45
Q

Which HLA is MMP associated with

A

HLA DQ7, DQB1*0301

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46
Q

MMP H&E vs BP

A

MMP: subepidermal blister with usually fewer eosinophils in the cutaneous lymphochistiocytic infiltrate than in bullous pemphigoid. Later stage: fibrosis

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47
Q

All patients with MMP will have +ve DIF

A

false

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48
Q

Most commonly +ve DIF site for MMP

A

Conjunctiva

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49
Q

T/F in mmp SSS increases sensitivity of autoantibodies and is more likely to detect IgA

A

T

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50
Q

MMP - genitals involved in half of female patients

A

t

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51
Q

2 types of skin lesions in MMP `

A

Generalised blistering (looks like BP) or localised blisters on an erythematous plaque

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52
Q

% sites involved in MMP

A

85% oral
Skin 25-30%
Genitals of females in 50%
Conjunctiva

53
Q

Pemphigoid gestationis occurs when

A

Pregnancy, hydatidiform mole, choriocarcinoma. Male more likely HLA-DR2

54
Q

Pemphigoid gestationis - target antigens

A

BP180 >BP230

IgG and C3 (as IgG1 avidly binds C3, C3 is sometimes more often detected than the IgG themselves)

55
Q

Pemphigoid gestationis - other associations

A

14% other autoimmune disease
Graves 10%
Hypothyroidism
vitiligo, alopecia, autoimmune thrombocytopenia

56
Q

Linear IgA antigens

A

BP180 (shed ectodomain LAD1) , BP230, LAD285 Collagen VII

57
Q

Least satisfactory DIF site for linear IgA

A

forearm

58
Q

t/f mucosal biopsies but not necessarily the conjunctiva are positive in linear IgA

A

t

59
Q

SSS with linear IgA usually shows the binding to the dermal side

A

F epidermal> dermal (dermal more likely collagen VII)

60
Q

IIF in linear IgA is more commonly +ve in adults than children t/f

A

F children 80% vs 30% adults

61
Q

Linear IgA - how to do IIF

A

Can use blister fluid, or serum

62
Q

T/F in linear IgA, IIF can use urine

A

F

63
Q

In chronic bullous disease of childhood, the mucosa is rarely involved

A

F - common. However if conjunctiva scarring - consider this to be MMP

64
Q

What age does chronic bullous disease of childhood usually occur?

A

Less than 5

65
Q

Associations with linear IgA

A

Drugs: vancomycin, frusemide, penicillin, diclofenac, lithium
Infection, IBD
Presence of building work in home, (50% adults and 75% children)
Increase in lymphoproliferative disease in adults. Case reports of increased risk of bladder and renal cancer

66
Q

Prognosis of linear IgA

A

Spontaneous remission after 3-6 years. Often improvement in pregnancy, but relapse post partum. No fetal problems

67
Q

Treatment of linear IgA

A

Dapsone

68
Q

Which type of EBA has the best prognosis

A

BP like

69
Q

Bullous SLE - target antigen

A

Collagen VII

70
Q

H&E bullous SLE

A

subepidermal blister with neutrophlic infiltrate, occasionally resulting in microabscesses resembling DH.

71
Q

DIF bullous SLE

A

Linear bands of IgG, IgA, IgM and C3

72
Q

T/F bullous SLE occurs when SLE flares

A

F

73
Q

SSS bullous SLE

A

Floor

74
Q

Tx of bullous SLE

A

Capstone, MTX, rituxumab

75
Q

DH: family hx of coeliac disease or DH in what %

A

10%

76
Q

DH: HLA associations

A

HLA DQ2, HLADR3, DQw2, HLAB8

77
Q

DIF DH

A

Granular IgA in papillary dermis

78
Q

IIF in DH

A

Negative for BMZ or dermal autoantibodies. But if monkey oesophagus used - IgA endomysial abs may be detected.

79
Q

Usual onset of DH

A

20-55

80
Q

T/F oral lesions do not occur in DH

A

F - common but asymptomatic

81
Q

Associations with DH

A

Gluten
Thyroid disease in 30%
Lymphoma (GI)
DM, SLE, vitiligo, sjogrens

82
Q

Prognosis of DH

A

10% remission

Do not have a reduced life expectancy, despite increased risk of lymphoma, likely due to reduced IHYD

83
Q

Tx of DH

A 
dapsone
sulfapyridine
Topical steroids
Heparin +/-tetracyclines + nicotinamide 
Gluten free diet 
Avoid iodine products
Gluten free after 2 years, maybe be able to reduce dapsone and diet after 5-10 years, protects from lymphoma.
84
Q

% of PNP with an existing neoplasm

A

2/3

85
Q

PNP IIF is +ve in what sort of epithelial

A

Simple, transitional and stratifying.

86
Q

Associations with PNP

A

40% NHL, 30% CLL, 10% castlemans, 6% thymomas, 6% sarcomas, 6% waldenstroms macroglobulinaemia

87
Q

Clinical features of PNP

A

Intractable stomatitis, pseudomembranous conjunctivitis, oesophageal, nasopharyngeal, vaginal, labia, penile, mucosal lesions. EM like lesions palms and soles.

88
Q

H&E of PNP

A

Suprabasal acantholysis and individual keratinocytes necrosis with lymphocytes in the epidermis. Basal cell liquefactive degeneration.

89
Q

PNP DIF

A

Intercellular and subepidermal IgG + C3.

90
Q

PNP target antigens

A

Plains (desmoplakin, envoplakin, BP230, periplakin. ) and desmogleins

91
Q

Complications of PNP

A

bronchiolitis obliterans - RFTs and CXray or CT

92
Q

T/F the intracellular location of plakin proteins makes it unlikely that antiplakin autoabs initiate pathology in PNP because IgG cannot penetrate cell membranes.

A

T

93
Q

After castlemans or thymoma removed, how long will it take to clear completely (PNP)

A

6-18/12

94
Q

Drug induced pemphigus - mechanism

A

thiol: provoke acantholysis by increasing the activity of plasminogen activators.
Non thiol: active amide group

95
Q

% of drug induced pemphigus that remits

A

40-50% thiols when drug withdrawn, only 15% non thiol group

96
Q

Thiol drugs (sulfhydryl group)

A

Captopril, penicillamine

97
Q

Non thiol causes of pemphigus

A

angiotensin II blockers, enalapril, ramapril, nifedipine, penicillin, cephalosporin, rifampicin, montelukast, hydroxychloroquine.

98
Q

Susceptibility allele to drug induced pemphigus

A

HLADR4

99
Q

Non drug triggers of pemphigus

A

allium family of veggies (onion, garlic, shallot, leek)

Radiotherapy, thermal burns and electrical injury

100
Q

Where does fogo selvagem occur?

A

Brazil, Columbia, el Salvador, Paraguay and Peru, Tunisia and Libya

101
Q

bg prevalence of antidesmoglein 1 abs in general population of endemic pemphigus

A

20% antidesmoglein 1 with non pathogenic subclasses or a non pathogenic domain of dsg1

43% have antidesmoglein 3 but significance is unknown

102
Q

Risk factor for endemic PF

A

Black flies at the end of the rainy season. Simuliidae bites

103
Q

Age group affected by endemic PF

A

Children and young adults.

104
Q

Endemic PF - sites affected and clinical features

A

head and neck first. Burning sensation (wild fire), spreads aurally and may become generalised.

105
Q

Endemic PF is often transferred via the placenta T/F

A

F - although the abs transfer, rarely does the disease occur in neonates. Desmoglein3 is more highly expressed in neonatal than adult skin and this may be protective.

106
Q

World wide PV is more common than PF

A

T

107
Q

Areas of the world where PF is more common than PV

A

Mali, Libya, south Africa.

108
Q

DIF PF

A

Intercellular IgG and C3, may be restricted to upper epidermis.

109
Q

IIF in PF = %

A

85%

110
Q

Areas which PF tends to occur

A

seborrhoeic areas - scalp, face, chest, upper back.

111
Q

Clinical variants of PF

A 
Pemphigus resembling DH (pemphigus herptiformis)
Pemphigus erythematosus(senear usher)
112
Q

senear usher DIF

A

Granular IGG and C3 at BMZ, intercellular IGG and C3 in epidermis

113
Q

Tx of PF

A 
Potent or IL steroids
Prod 20-40mg/day 
aza, cyclophosphamide
HCQ as adjuvant 
IVIg
MTX
Dapsone 
MMF
Nicotinamide 1.5mg + minocycline 100mg daily 
Rituximab.
114
Q

H&E PF

A

Dyskeratotic cells in the granular layer of older lesions with a mixed inflammatory infiltrate of eosinophils and Neutrophils in the superficial dermis.

115
Q

T/F people with PV in India tend to be older than those elsewhere

A

F younger

116
Q

Types of pemphigus

A 
PV - variant: veterans
PF - vary: PH, PE
Drug induced
Intercellular IGA 
PNP
117
Q

% of pemphigus that is PV

A

70%

118
Q

Races that more susceptible to PV

A

Ashkenazi Jews, Indians, eastern countries

119
Q

HLA and PV

A

DRB10402 and DRB11401

120
Q

IN Iranian pemphigus, epidemiology

A

Increase in OCP, pesticides, protective effect from smoking

121
Q

In PV patients, Indian patients are more likely to have what

A

Dsg 1

122
Q

Clinical features PB

A

Oral lesions 50-70%
Scalp, face, axillae, groins and pressure points.
Nail dystrophy and paronychia

123
Q

PV - poor prognostic factors

A

Jews, older patients

124
Q

PV, most relapse occur when

A

First 2 years after Dx.

125
Q

2 subtypes of pemphigus vegetans

A

Neumann (severe) and hallopeau (mild)

126
Q

Pemphigus veterans antigens

A

desmocollins 1 and 2 and dig 3

127
Q

2 types of IgA pemphigus

A

1) subcorneal pustular (desmocollin 1) - associated with monoclonal IgA gammopathy
2) intraepidermal neutrophilic type (sometimes desmocollin1)

128
Q

Associations with IgA pemphigus

A

IgA monoclonal gammopathy, HIV, IBD, gluten sensitive enteropathy, RA, thiol drugs

Blistering disorders Melissa (1 decks)

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