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BDS5 oral disease > autoimmune blistering conditions > Flashcards

autoimmune blistering conditions Flashcards

1
Q

describe bullous pemphigoid (what, target, age)

A
  • acquired autoimmune blistering disease causing subepidermal split
  • BP180, BP230
  • ≥70yo
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2
Q

signs and symptoms of bullous pemphigoid (3)

A
  • <30% with oral lesions
  • pruritus
  • tense intact blisters with background erythema
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3
Q

treatment of bullous pemphigoid (3)

A
  • topical corticosteroids
  • dapsone/tetracycline (antibiotics)
  • prednisolone, azathioprine, mycophenolate
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4
Q

bullous pemphigoid age group

A

≥70yo

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5
Q

describe mucous membrane pemphigoid (what, targets, demographic)

A
  • acquired autoimmune disease characterised by blistering of mucous membranes
  • BP180, laminin 332 (possibly BP230, integrins, collagen VII)
  • older females (60-70yo)
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6
Q

mucous membrane pemphigoid antigens (up to 5)

A

BP180 and laminin 332
possibly BP230, integrins, collagen VII

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7
Q

mucous membrane pemphigoid demographic

A

older females 60-70yo

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8
Q

oral s/s of MMP (2)

A
  • desquamative gingivitis
  • yellow ulcers with background erythema
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9
Q

management of MMP (5)

A
  • topical as per RAS +/- intralesional triamcinolone
  • dapsone
  • hydroxychloroquine
  • if severe = prednisolone + azathioprine/mycophenolate/rituximab
  • vaccines needed pre-tx
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10
Q

describe pemphigus vulgaris (what, targets, demographic)

A
  • acquired autoimmune mucocutaneous blistering disease causing suprabasal acantholysis
  • desmoglein 3 +/- 1
  • 40-60yo female, Asian, Jewish
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11
Q

pemphigus vulgaris demographic

A

middle aged females 40-60yo
Asian and Jewish

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12
Q

s/s of pemphigus vulgaris (7)

A
  • fragile bullae/erosions on normal background mucosa/skin
  • +/- peeling, flaking, crusting
  • acute paronychia
  • bright red eyes
  • perioral erosions and secondary infection
  • oral erosions/excoriations, ragged friable gingivae
  • positive Nikolsky’s sign
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13
Q

what is positive Nikolsky’s sign? (2)

A
  • sign of blistering condition
  • shearing pressure on skin/mucosa = layer of skin will shear off
    (very tender and not recommended)
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14
Q

pemphigus vulgaris antigen

A

desmoglein 3 +/- 1

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15
Q

pemphigus vulgaris DIF result

A

intercellular IgG and C3

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16
Q

pemphigus treatment (3)

A
  • topical corticosteroids
  • oral high dose prednisolone and steroid-sparing agents (azathioprine, mycophenolate)
  • rituximab if resistant
17
Q

describe pemphigus foliaceous (what, age, presentation)

A
  • acquired autoimmune blistering disease affecting SKIN only
  • 50-60yo
  • superficial scaling +/- erosions
  • face/neck/upper trunk
18
Q

describe paraneoplastic pemphigus (what, age)

A
  • mucocutaneous blistering associated with an underlying malignancy
  • 45-70yo
19
Q

three main types of pemphigus

A

pemphigus vulgaris
pemphigus foliaceous
paraneoplastic pemphigus

20
Q

two main types of pemphigoid

A

bullous pemphigoid
mucous membrane pemphigoid

21
Q

s/s of paraneoplastic pemphigus (3)

A

periocular and perioral lesions, flexural areas
- panstomatitis (deep, red)
- ulcerated lesions with yellow slough +/- corrugated appearance (hyperplasia)

22
Q

describe linear IgA bullous disease (what, cause, age, targets, characteristic lesion)

A
  • acquired autoimmune blistering disease, 25% with oral lesions
  • triggered by infection or penicillins
  • <5yo or >50yo
  • BP180 and collagen VII
  • cluster of blisters in a ring
23
Q

linear IgA bullous disease age group

A

two peaks <5yo or >50yo

24
Q

linear IgA bullous disease antigens (2)

A

BP180 and collagen VII

25
Q

linear IgA bullous disease characteristic lesion

A

cluster of blisters in a ring

26
Q

describe epidermolysis bullosa acquisita (what, age, target)

A
  • acquired equivalent of dystrophic EB (congenital)
  • severe mucocutaneous subepidermal blistering disease
  • middle aged adults
  • type VII collagen
27
Q

congenital equivalent of epidermolysis bullosa acquisita

A

dystrophic EB

28
Q

epidermolysis bullosa acquisita antigen

A

collagen VII

29
Q

epidermolysis bullosa acquisita s/s (4)

A

deep blisters leading to scarring:
- loss of nails
- microstomia
- ankyloglossia

30
Q

describe dermatitis herpetiformis (what, age)

A
  • acquired autoimmune blistering disease associated with gluten sensitivity/coeliac disease
  • IgA deposits in skin
  • 20-40yo
31
Q

dermatitis herpetiformis age group

32
Q

dermatitis herpetiformis s/s (2)

A

itchy rash on extensor surfaces
rarely oral ulceration

33
Q

dermatitis herpetiformis treatment (2)

A

dapsone and gluten-free diet

BDS5 oral disease (26 decks)

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