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Oral Pathology II > 16: Immune-Mediated Mucocutaneous Diseases > Flashcards

16: Immune-Mediated Mucocutaneous Diseases Flashcards

1
Q

Pemphigus Vulgaris: Overview

A

Autoimmune

Autoantibodies destroy desmosomes

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2
Q

Pemphigus Vulgaris: Population

A

Adults, 30s-50s

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3
Q

Pemphigus Vulgaris: Clinical

A

50% oral lesions “First to show, last to go”
Ragged erosions + ulcerations
Flaccid bullae on skin, none intact in mouth
Positive Nikolsky sign

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4
Q

Pemphigus Vulgaris: Immunopathologic

A 
Positive DIF (and IIF)
Autoantibodies bind desmosomal components (desmoglein 3 & 1)
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5
Q

Pemphigus Vulgaris: Histopathology

A

Intraepithelial (aka suprabasilar) clefting
Acantholysis = breakdown of spinous layer
“Dilapidated brick wall” with intact basal cell layer

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6
Q

Pemphigus Vulgaris: Treatment

A

Systemic (not topical) corticosteroids

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7
Q

Pemphigus Vulgaris: Prognosis

A

Fatal if not treated

Now 5-10% mortality due to complications

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8
Q

Mucous Membrane Pemphigoid: Overview

A

aka Cicatricial Pemphigoid
Blisters resemeble pemphigus
2-4x more common than pemphigus

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9
Q

Mucous Membrane Pemphigoid: Population

A

2:1 female, 50-60 yo

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10
Q

Mucous Membrane Pemphigoid: Clinical Presentation

A

Any mucosal surface
Scarring with conjunctival and cutaneous lesions
Desquamative gingivitis
Intact intraoral blisters

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11
Q

Mucous Membrane Pemphigoid: Ocular Involvement

A

Significit

Scarring –> symblepharons = adhesion formation between eyelid and globe

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12
Q

Mucous Membrane Pemphigoid: Histopathology

A

Clean subepithelial cleft

Separates intact epi at CT from BMZ

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13
Q

Mucous Membrane Pemphigoid: Immunopathology

A

Normal mucosa (.5-1cm from ulceration/erythema)
Submitted in Michel’s solution and formalin
Linear deposition of immuinoreactants at BMZ
Positive DIF, negative IIF

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14
Q

Mucous Membrane Pemphigoid: Treatment

A

Depends on involvement
Oral: topical corticosteroids
Ocular: systemic immunosuppressive

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15
Q

Mucous Membrane Pemphigoid: Prognosis

A

Rarely fatal
Ocular –> blindness
Rare spontaneous resolution

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16
Q

Bullous Pemphigoid: Population

A

Older, 75-80

17
Q

Bullous Pemphigoid: Clinical Presentation

A

Mostly cutaneous lesions, 10-20% oral

Pruritus, then cutaneous blisters

18
Q

Bullous Pemphigoid: Histopathology

A

Subepithelial cleft (like MMP)
Positive DIF + IIF
Immunoreactants deposited at BMZ

19
Q

Bullous Pemphigoid: Treatment

A

Like MMP

Immunosuppressive can have serious SE

20
Q

Bullous Pemphigoid: Prognosis

A

Can spontaneously resolve 1-2 years

21
Q

Erythema Multiforme: Overview

A

Acute, self-limited ulcerative disorder
Immune mediated
50% unknown
50% infection, medication

22
Q

Erythema Multiforme: Population

A

Young adult female

23
Q

Erythema Multiforme: Disease Spectrum

A

EM minor: skin, oral mucosa only
EM major: 2+ mucosal sites + skin
Steven’s Johnson Syndrome: 30% body sites

24
Q

Erythema Multiforme: Clinical Features

A

Hemorrhagic lip crusting
Oral ulcers with ragged margins
“Target” skin lesions

25
Q

Erythema Multiforme: Sites

A

Labial + buccal mucosa, tongue

26
Q

Erythema Multiforme: Histopathology

A 
Characteristic, not diagnostic
Keratinocyte destruction
Subepithelial edema
Mixed inflammatory infiltrate
Perivascular inflammation
Non-specific DIF
27
Q

Erythema Multiforme: Treatment

A

EM minor: supportive
EM major: corticosteroids (controversial)
TEN: IV pooled human Ig

28
Q

Erythema Multiforme: Prognosis

A

20% cases recur
EM major: 1-5% mortality
TEN: 25-30% mortality, but now improved

29
Q

Erythema Migrans: Overview

A

aka Bengin Migratory Glossitis, Geographic Tongue
Common, 1-3%
Immune-mediated

30
Q

Erythema Migrans: Clinical

A

With fissured tongue
Lesions wax + wane
Ectopic geographic tongue = on non-keratinized mucosal surfaces

31
Q

Erythema Migrans: Histopathology

A

Biopsy shows “psoriasiform mucositis”
Parakeratosis with microabscess in upper spinous layer
Lose superficial keratin –> thins –> red

32
Q

Erythema Migrans: Treatment

A

None

Topical corticosteroid gel if sensitive

Oral Pathology II (3 decks)

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