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Immune-Mediated Diseases Affecting Oral Mucosa Flashcards

1
Q

Recurrent apthous stomatitis is a synonym for…

A

“canker sores”

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2
Q

Painful ulcers with red halo

A

Recurrent aphtous stomatitis, or “canker sores”

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3
Q

___% of the population are afflicted with canker sores

A

20%

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4
Q

Pathogenesis of recurrent aphthous stomatitis

A
  • common manifestation of many disease processes, each mediated through immune system
  • epithelial destruction due to TNF-alpha generated by T cells, macrophages and mast cells
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5
Q

Epithelial destruction in recurrent aphthous stomatitis is due to…

A

TNF-alpha generated by T-cells, macrophages, and mast cells

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6
Q

Proposed etiologic factors of recurrent aphthous stomatitis

A
  • Allergies
  • Nutritional deficiences
  • Genetic predisposition
  • Hematologic factors
  • Hormonal factors
  • Infections
  • Stress
  • Trauma
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7
Q

3 categories of proposed etiologic factors

A
  • Primary immunodysregulation
  • Decreased mucosal barrier
  • Increased antigenic exposure
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8
Q

Minor aphthous ulcers make up what percentage of RAU cases?

A

> 80% RAU cases

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9
Q

Major aphthous ulcers make up what percentage of RAU cases?

A

~10% RAU cases

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10
Q

Size of minor aphthous ulcer vs major aphthous ulcers

A
  • Minor is < one cm in diameter

* Major is larger

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11
Q

Shape of minor aphthous ulcer vs major aphthous ulcers

A
  • Minor is symmetrical

* Major has an irregular shape

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12
Q

Timeline of resolution of a minor and major aphthous ulcer

A
  • Minor resolves in 2 weeks

* Major persists up to 6 weeks

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13
Q

Healing outcome of minor vs major aphthous ulcer

A
  • Minor aphthous ulcers heals w/o scarring

* Major aphthous ulcers heals with scarring

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14
Q

Quantity of lesions in minor and major aphthous ulcers

A
  • Minor has fewer lesions than in major

* Major has more lesions than in minor

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15
Q

Location of minor and major aphthous ulcers

A
  • Minor occurs in lining mucosa

* Major occurs most in lining mucosa

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16
Q

Minor aphthous ulcers

A
  • Prodromal burning or itching
  • Erythematous macule
  • Fibrinopurulent membrane with red halo
  • 1-5 lesions/episode
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17
Q

Major aphthous ulcers are also called…

A
  • Sutton’s Disease

* Periadenitis Mucosa Necrotica Recurrens

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18
Q

Major aphthous ulcers

A
  • Deeper and more painful than minor form
  • Any oral site but more on lining mucosa
  • 1-10 lesions/episode
  • DDx includes squamous cell carcinoma
  • Scarring rarely –> restricted mouth opening
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19
Q

Herpetiform aphthous ulcers

A
  • Least common type
  • 1-3 mm
  • < 100 ulcers/episode
  • Heal 7-14 days
  • Most frequent recurrences
  • Any oral site, but more common on lining mucosa
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20
Q

Simple aphthosis

A
  • Few lesions
  • Heal in 2 weeks
  • Few recurrences
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21
Q

Complex aphthosis

A
  • Multiple lesions
  • Rapid recurrences
  • Severe pain
  • +/- genital or perianal lesions
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22
Q

Diagnosis of recurrent aphthous ulcers

A
  • Clinical criteria
  • Histology: non-specific ulcer
    • Biopsy excludes other diagnoses
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23
Q

The following conditions must be ruled out in the diagnosis of recurrent aphthous ulcers…

A
  • Hematologic deficiency
  • GI disease
  • Immunodeficiency
  • Drug reaction
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24
Q

Management of simple aphthosis

A

Topical treatment:
• OTC anesthetics
• Topical corticosteroids

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25
Q

Management of complex aphthosis

A

Rule out systemic disease:
• More potent topical corticosteroids
• Injected corticosteroids
• Cyclic neutropenia

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26
Q

Disorders associated with oral aphthous ulcers

A
  • Behcet’s syndrome
  • Reiter’s syndrome
  • Immunocompromised conditions (HIV)
  • CIBD (Crohn’s & Ulcerative colitis)
  • Nutritional deficiencies
  • Celiac disease
  • Cyclic neutropenia
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27
Q

Behcet’s syndrome (disease)

A
  • “Chronic ocular inflammation + orogenital ulcers”
  • Actually multisystem disease
  • Immunodysregulation
  • Possible genetic predisposition
  • Rare in most countries
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28
Q

Environmental antigens that trigger Behcet’s Syndrome

A
  • Bacteria (streptococci)
  • Viruses
  • Pesticides
  • Heavy metals
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29
Q

Histocompatibility antigen associated with Behcet’s syndrome

A

Histocompatibility antigen B-51 (HLA-B51) link

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30
Q

Common in Japan, Middle East

A

Behcet’s and HLA-B51

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31
Q

Diagnosis of Behcet’s Syndrome

A 
• Oral aphthous ulcers - 3 recurrences per year
• PLUS 2 of 4:
     - Genital lesions
     - Ocular lesions
     - Cutaneous lesions
     - Pathergy
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32
Q

Histology of Behcet’s Syndrome

A

• Similar to aphthous ulcers
- T lymphocytes prominent
• Leucoytoclastic vasculitis
- Neutrophil attack on small artery walls
- Karyorrhexis of neutrophils
- Fibrinoid: smudy fibrin-like deposits of degenerated collagen or ground sub in arterial walls

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33
Q

Treatment for Behcet’s Syndrome

A 
• Corticosteroids
• Immunomodulators
     - Imuran 
     - Cyclophosphamide
     - Cyclosporine
     - Methotrexate
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34
Q

Prognosis of Behcet’s Syndrome

A 
• Variable
• Fewer attacks
• Prognosis good unless:
     - CNS disease
     - Severe vascular changes
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35
Q

Common chronic skin condition (affects approximately 1.5 million Americans)

A

Psoriasis

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36
Q

Etiology of psoriasis

A

• Immunologic factors
- TNF etc. causes keratinocyte proliferation
• Lesions coincide with T-cell infiltration into epidermis
• Genetic factors

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37
Q

Clinical features of Psoriasis

A

• Starts age 10 to 30 years
• Persists for years
• Worse in winter
• Symmetrical distribution on scalp, elbows, knees
• Well-defined erythematous plaques with silvery scale
- May itch, be painful, and can bleed
• Arthritis in 10%
• Oral lesions uncommon
- Red plaques
- Erythema migrans?

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38
Q

Well-defined erythematous plaques with silvery scale are observed in…

A

Psoriasis

Note: it may itch, be painful, and can bleed

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39
Q

Psoriasiform histologic pattern

A
  • Parakeratosis
  • Microabscesses
  • Long rete ridges
  • High connective tissue papillae
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40
Q

Psoriasis differential diagnosis

A
  • Erythema migrans
  • Reiter’s syndrome
  • Candidiasis
  • Psoriasis
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41
Q

Synonyms for Erythema Migrans

A
  • Geographic tongue
  • Benign migratory glossitis
  • Erythema areata migrans
  • Psoriasiform mucositis
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42
Q

Clinical features of erythema migrans

A
  • Multiple well-defined lesions
  • Erythematous zone
  • White or yellow slightly raised border
  • +/- burning sensation
  • Anterior 2/3 dorsal tongue > lining mucosa
  • Atrophic filiform papillae
  • Lesions enlarge, subside, appear elsewhere
  • +/- fissured tongue
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43
Q

Demographics of erythema migrans

A
  • Common
  • F > M
  • <3% of population
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44
Q

Etiology of erythema migrans

A

• Etiology unknown
• Possible hypersensitivity reaction
• Erythema migrans + psoriasis associated with HLA-Cw6?
- Similar histology

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45
Q

Histology of erythema migrans

A

Periphery:
• Hyperkeratosis
• Acanthosis
• Elongated rete ridges

Center:
• Intraepithelial microabscesses (neutrophils)

Lamina propria
• Acute and chronic inflammatory cells

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46
Q

Management of erythema migrans

A
  • “Reassurance”

* Topical corticosteroids

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47
Q

Demography of lichen planus

A
  • Middle age onset

* F slightly > M

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48
Q

What is lichen planus?

A
  • Chronic immunologically-mediated disease
  • Oral lesions +/- lesions on skin, other mucosae
  • Oral lesions more persistent than skin lesions
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49
Q

Skin lesions of lichen planus

A
  • Most on flexor surfaces
  • Plaques or papules
  • Purple, flat-topped with white (Wickham’s) striae
  • Pruritic
  • Wax and wane and often subside in 2 years
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50
Q

Types of oral lesions in lichen planus

A
  • White adherent
  • Erosive
  • Vericulobullous
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51
Q

White adherent oral lesion of lichen planus

A 
• Asymptomatic
• Reticular striae
     - Interlacing white lines
     - Bilateral and symmetrical
     - Buccal mucosa > tongue > gingivae
• Plaques
     - Tongue and buccal mucosa
• Papules
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52
Q

Erosive (e.g. desquamative gingivitis) oral lesion of lichen planus

A
  • Symptomatic
  • Atrophic/Erythematous areas around central ulceration
  • Ulcerations of varying degrees
  • +/- peripheral white striae
  • very rare malignant transformation
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53
Q

Which type of oral lesion of lichen planus is rare?

A

Vesiculobullous

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54
Q

Histology of lichen planus

A

• Orthokeratosis or parakeratosis
- Thickness of keratin correlates with clinical appearance
• Uneven acanthosis
• Rete ridges prominent, sharp (“saw tooth”), or absent
• T-lymphocyte zone in upper lamina propria
• Loss of basal cells
• Colloid, cytoid or Civette bodies

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55
Q

What are Civette bodies?

A

Apoptotic basal cells are eosinophilic degenerating keratinocytes

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56
Q

When are Civette bodies observed?

A

Lichen planus

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57
Q

What’s observed in the immunofluorescence of Lichen planus?

A
  • Nonspecific: shaggy fibrinogen band along BMZ

* Globular deposits of IgM and complement on colloid bodies in some cases

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58
Q

Clinical differential diagnosis for Lichen Planus

A 
• Hypersensitivity reactions
     - Systemic (e.g. drugs)
     - Local or contact (e.g. amalgam, cinnamon)
• Lupus erythematosus
• Chronic ulcerative stomatitis
• Oral graft-versus-host disease
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59
Q

Management of Lichen Planus

A

• Reticular
- No treatment needed after diagnosis
- Clinical monitoring
• Erosive
- Topical corticosteroids
- Systemic immunosuppressives if necessary
- Monitor for potential dysplasia, SCC
- Monitor for iatrogenic candidiasis

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60
Q

What is Lichenoid Mucositis?

A
  • Lesions with clinical and/or histological resemblance to classical lichen planus
  • May exhibit some variation from classical features
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61
Q

Types of pemphigus

A
  • Vulgaris
  • Vegetans
  • Foliaceus*
  • Erythematosus*
  • Paraneoplastic
  • Drug induced
  • Do not affect oral cavity
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62
Q

Which types of pemphigus do not affect oral cavity?

A
  • Foliaceus

* Erythematosus

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63
Q

Clinical features of pemphigus vulgaris

A
  • Autoimmune vesiculobullous mucocutaneous disease
  • Appears first in mouth in some cases
  • Childhood to old age, but most 30-50 years
  • Flaccid lesions
  • Nikolsky sign positive
  • Painful
  • Vesicles rupture rapidly to form irregularly-shaped ulcers as they spread peripherally and coalesce
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64
Q

What is a positive Nikolsky sign?

A

Firm lateral pressure on normal-appearing mucosa (or skin) produces a blister

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65
Q

Pemphix (Greek) means…

A

bubble or blister

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66
Q

Nikolsky sign positive in many cases of pemphigus and also in percentage of some other mucocutaneous conditions including…

A
  • Pemphigus vulgaris
  • Paraneoplastic pemphigus
  • Pemphigoid (all types)
  • Bullous lichen planus
  • Erythema multiforme
  • Hypersensitivity reactions
  • Epidermolysis bullosa
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67
Q

Histology of pemphigus vulgaris

A

• Acantholysis
• Suprabasilar cleft with tombstone basal cells
- Cleft contains Tzanck cells

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68
Q

Diagnosis of pemphigus vulgaris

A
  • Exfoliative cytology: acantholytic round epithelial (Tzanck) cells
  • Perilesional biopsy
  • Direct immunofluorescence: labeled Igs attach** to autoantibodies to desmogleins 1 and 3 arund epithelial cells in biopsy specimen. This creates a fishnet pattern.
  • Indirect immunofluorescence: labelled circulating** autoantibodies to desmoglein 1 and 3 create same pattern on animal mucosa
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69
Q

Management of pemphigus vulgaris

A
  • Diagnose as soon as possible
  • Rule out drug reaction
  • Medication should be prescribed by experienced physician
  • Systemic corticosteroids
  • Other immunosuppressive agents
  • Monitor for iatrogenic candidiasis
  • Monitor disease by I.I.F.
  • 10% fatal due to medications
70
Q

Drug reactions related to pemphigus…

A
  • A variety of drugs have been implicated in the onset of drug-induced pemphigus
  • Some of these drugs induce antibody formation, which results in acantholysis via a mechanism identical to that found in idiopathic pemphigus.
  • Other drugs are posulated to induce acantholysis directly in the absence of antibody formation
71
Q

Side effects of systemic corticosteroids in pemphigus…

A
  • Diabetes mellitus
  • Adrenal suppression
  • Weight gain
  • Osteoporosis
  • Peptic ulcers
  • Severe mood swings
  • Increased susceptibility to infections
72
Q

Circulating Igs correlate with disease activity in…

A
  • I.I.F. used to monitor disease

* Pemphigus

73
Q

Pemphigus vegetans

A

• Variant of pemphigus vulgaris
• Oral involvement in a few cases
• Acantholytic bullae are followed by epithelial hyperplasia and intraepithelial abscess
- Eosinophils within the abscesses
• Pustular vegetations may look verrucous
• Vegetans type may occur in lull in pemphigus vulgaris
• Can spontaneously remit

74
Q

Mucocutaneous disease associated with lymphoma or benign lymphoproliferative disease

A

Paraneoplastic pemphigus

75
Q

May appear before lymphoma is diagnosed…

A

paraneoplastic pemphigus

76
Q

Clinical presentation of paraneoplastic pemphigus

A
  • Sudden onset of multiple vesiculobullous lesions on skin and mucosae
  • Cicatricial conjunctivitis in some cases
  • Skin lesions papular and pruritic (like lichen planus)
  • Lips resemble erythema muliforma (crusting)
77
Q

Corticosteroid controls this disease but makes malignancy worse…

A

Paraneoplastic pemphigus

78
Q

Pathogenesis of paraneoplastic pemphigus

A
  • Igs against intercellular and basement membrane zone antigens
  • Tumor –> host lymphocytes –> IL-6
  • Cytotoxic T lymphocytes
  • This multifaceted immunologic attack produces variety of clinical, histological & immunological changes
79
Q

In paraneoplastic pemphigus, IL-___ stimulates Igs against…

A

IL-6 stimulates Igs against:
• Desmosomal antigens
• Basement membrane zone antigens

80
Q

Histology of paraneoplastic pemphigus

A

• Lichenoid mucositis with subepithelial cleft or intraepithelial cleft
- Some cases are only lichenoid
• Immunoprecipitation studies: antibodies against desmoglein 1 and 3, desmoplakin I and II, BPAG1

81
Q

Immunoprecipitation studies in paraneoplastic pemphigus show antibodies against…

A
  • Desmoglein 1 and 3
  • Desmoplakin I and II
  • BPAG1
82
Q

Demographic of mucous membrane pemphigoid

A

Most often affects females, especially those over 40 years of age

83
Q

Mucous membrane pemphigoid clinical features

A
  • Chronic vesiculobullous disease
  • Vesicles rupture: painful ulcers may persist for months
  • One cause of desquamative gingivitis
  • Conjunctival involvement may –> scarring
84
Q

Nikolsky sign for mucous membrane pemphigoid

85
Q

Where can mucous membrane pemphigoid occur?

A

Any part of oral mucosa, other mucosae, (skin)

86
Q

Describe the conjunctival involvement in mucous membrane pemphigoid

A

Conjunctival involvement may –> scarring:
• Symblepharon: adhesions between bulbar and palpebral conjunctivae
• Ankyloblepharon: adhesion of ciliary edges of eyelids to each other
• Entropion: scarrying may –> eyelids turn inwards

87
Q

Pathogenesis and histology of mucous membrane pemphigoid

A
  • Autoantibodies (IgG) to BMZ antigens
  • Binding of IgG triggers a reaction involving complement and neutrophils
  • Weakens basement membrane
88
Q

Discuss autoantibodies to BMZ antigens involved in mucous membrane pemphigoid

A

Hemidesmosome:
• BPAG1 (230kd) in plaque
• BPAG2 (180kd) transmembrane protein
• Integrin α6β4

Laminda lucida:
• Laminin (epiligrin) in anchoring filaments

89
Q

Immunofluorescence of mucous membrane pemphigoid

A

• Homogenous linear fluorescence at basement membrane zone on D.I.F.
- IgG, C3, etc.
• Circulating Ig’s (I.I.F.) in only 5-30% cases

90
Q

Differential diagnosis of mucous membrane pemphigoid (subepithelial vesicles in oral vesiculo-ulcers)

A
  • Pemphigoid (all types)
  • Epidermolysis bullosa (some types)
  • Linear IgA disease
  • Angina bullosa hemorrhagica
  • Dermatitis herpetiformis
91
Q

Management of mucous membrane pemphigoid

A 
• Rule out drug induced disease
• Ophthalmic consult
• Topical corticosteroids
     - Increase potency as necessary
• Systemic
     - Corticosteroids
     - Steroid sparing immunosuppressives
     - Tetracycline or minocycline
     - Dapsone
92
Q

What is desquamative gingivitis?

A
  • Gingivae red, edematous, and glazed
  • Superficial ulceration or desquamation/peeling
  • Buccal > lingual
93
Q

Desquamative gingivitis occurs in various vesiculobullous conditions including…

A
  • Mucous membrane pemphigoid
  • Erosive lichen planus
  • Chronic ulcerative stomatitis
  • Pemphigus vulgaris
  • Drug reactions
  • Epidermolysis bullosa acquisita
  • Systemic lupus erythematosus
  • Linear IgA disease
94
Q

Most common A-I blistering disease

A

Bullous pemphigoid

95
Q

Demography of bullous pemphigoid

A

Older people (60-80)

96
Q

Clinical presentation of bullous pemphigoid

A
  • Starts with pruritus
  • Multiple tense bullae on normal or erythematous skin
  • Bullae rupture producing crust
  • Heal w/o scars
97
Q

Clinical course of bullous pemphigoid in relation to mucous membrane pemphigoid

A

Clinical course shorter than MMP

98
Q

How common are oral lesions in bullous pemphigoid?

A

Oral lesions uncommon (~15%)

99
Q

Histology of bullous pemphigoid

A
  • Subepithelial cleavage

* Eosinophils within bullae

100
Q

Antigens involved in bullous pemphigoid

A

Antigens: BP 180 and BP 230

101
Q

Immunofluorescence for bullous pemphigoid

A
  • D.I.F. positive in 90% to 100%

* I.I.F. positive in 50% to 90%

102
Q

What do titers in bullous pemphigoid indicate about the disease activity?

A

Titers don’t correlate with disease activity.

103
Q

Management of bullous pemphigoid

A
  • R/o drug-induced disease
  • Systemic immunosuppressive agents
  • Lower doses of prednisone than for pemphigus
  • Better prognosis
  • Reported spontaneous remission in 2-5 years in some cases
  • Mortality due to treatment in older patients
104
Q

Pathogenesis of systemic lupus erythematosus

A 
• Genetic, environmental and hormonal factors trigger:
     - Increased B cell function
     - Abnormal T cell activity
• Antibodies against host cell antigens:
     - Nuclear
     - Cytoplasmic
     - Cell surface
105
Q

Clinical features of systemic lupus erythematosus

A
  • Young adult females
  • Erythematous cutaneous rash (“butterfly” pattern on face)
  • Fever, weight loss, malaise
  • Glomerulonephritis
  • Damage to: Joints, Heart, Lungs
106
Q

Erythematous cutaneous rash, or “butterfly” pattern on face is indicative of…

A

Systemic lupus erythematosus

107
Q

Oral lesions found in SLE

A
  • White plaques +/- ulceration
  • Erythema/Erosions/Ulcers
  • Desquamative gingivitis
  • Classical lesion: central red area or ulcer with white spots and peripheral radiating white lines
  • Oral lesions may occur in SLE and CCLE
108
Q

This classical lesion is associated with what? –> Central red area or ulcer with white spots and peripheral radiating white lines

A

Systemic Lupus Erythematosus

109
Q

Laboratory abnormalities observed in SLE…

A 
• Hematologic changes:
     - Anemia
     - Leukopenia (decrease in WBC)
     - Thrombocytopenia (decrease in thrombocytes)
• Reduced serum complement concentration
• Antinuclear antibodies (ANAs)
110
Q

What is the most common technique to detect antinuclear antibodies (ANAs) in SLE?

A

Indirect immunofluorescence

111
Q

What does the pattern of nuclear fluorescence indicate in SLE?

A

Pattern of nuclear fluorescence suggests the type of antibody.

112
Q

SLE: homogenous/diffuse pattern of nuclear fluorescence

A

antibodies to chromatin, histones, and ds-DNA

113
Q

SLE: rim pattern of nuclear fluorescence

A

antibodies to ds-DNA

114
Q

SLE: specked pattern of nuclear fluorescence

A 
Antibodies to non-DNA antigens (histones and RNP):
• Sm
• RNP
• SS-A (Ro)
• SS-B (La)
115
Q

SLE: nucleolar pattern of nuclear fluorescence

A

antibodies to nucleolar RNP

116
Q

Histology of SLE

A

• Hyperkeratosis
- Follicle keratin plugging in skin in CCLE (Chronic cutaneous lupus) but not in SLE
• Alternating epithelial atrophy/acanthosis
• Basal cell degeneration (apoptotic bodies)
• Subepithelial edema (+/- vesicles)
• Thick PAS-positive basement membrane zone
• Subepithelial vascular and adnexal lymphocytes

117
Q

Immunofluorescence results for SLE and CCLE

A

• I.F. shows shaggy, granular-linear deposits in band along mucocutaneous basement membrane zone

 - IgG (IgM & IgA), C3, and fibrinogen
 - Band along basement membrane zone is lupus band test
 - Positive in clinically normal skin in SLE (not in CCLE)
118
Q

Pathogenesis of systemic sclerosis

A

Probably autoimmune pathogenesis

119
Q

What is systemic sclerosis and its clinical presentation?

A
  • Increased collagen deposition
  • Insidious onset
  • Fibrosis of lungs, heart, kidneys, GI tract, skin
  • Interstitial pulmonary disease –> secondary pulmonary hypertension –> right-sided heart failure
  • Sclerodactyly: symmetrical thickening, tightening and induration of skin of fingers and toes
120
Q

Demography of systemic sclerosis

A

Females affected more than males

121
Q

Disease characterized by increased collagen deposition

A

Systemic sclerosis

122
Q

What is Raynaud’s phenomenon?

A

• Arterial insufficiency of acral parts secondary to another disorder that causes arterial narrowing:
- SLE, systemic sclerosis, etc.
• Claudication (limping), color and temperature changes
• Chronic ulcerations and eventual gangrene
• In Raynaud’s disease, vasospasm and its consequences are primary (increased response to stimuli has no known cause)

123
Q

Histology of systemic sclerosis…

A

Dense collagen replaces and destroys normal tissue

124
Q

Serology of Systemic Sclerosis

A

• Rheumatoid factors (RF) is antibody against Fc fragment of human IgG
• ANAs:
- Anti-Scl-70 (antibodies to topoisomerase I)
- Anticentromere antibodies
- No evidence that humoral immunity plays a significant role in pathogenesis, but these two ANAs are virtually unique to this disease and anti-Scl-70 is a marker for development of more aggressive disease

125
Q

What medication may inhibit collagen formation in systemic sclerosis?

A

D-penicillamine

126
Q

Additional management for systemic sclerosis

A

• Surgery
• Esophageal dilation
• Calcium channel blockers
- Increase peripheral blood flow and reduce Raynaud’s changes
• ACE (Angiotensin converting enzyme) inhibitors
- Reduce hypertension if kidneys severely affected
• Oral hygiene instruction
• Poor long-term prognosis

127
Q

What is Crest syndrome?

A

Mild form of systemic sclerosis

128
Q

Demography of crest syndrome

A

50-70 year old females

129
Q

Symptoms of crest syndrome

A
  • Calcinosis cutis
  • Raynaud’s phenomenon
  • Esophageal dysfunction
  • Sclerodactyly
  • Telangiectasia
130
Q

What is erythema multiforme?

A
  • Ulcerative mucocutaneous disease
  • “Self-limiting” hypersensitivity reaction
  • Acute, chronic, or recurring acute
131
Q

Types of erythema multiforme…

A 
• Erythema multiforme minor
• Erythema multiforme major
     - Stevens-Johnson syndrome
• Toxic epidermal necrolysis (TEN)
     - Lyell's disease
132
Q

Pathogenesis for erythema multiforme

A

• Uncertain
• Immunologically mediated
• Some immunologic precipitating/triggering events
- Infections: HSV, Mycoplasma pneumoniae

133
Q

Demography of erythema multiforme minor

A
  • Young adults

* Males affected more often than females

134
Q

Clinical presentation of erythema multiforme minor

A

Abrupt onset:
• Some cases chronic
• Some cases recurrent (linked to HSV)

Prodrome: fever, malaise, headache

135
Q

Oral lesions observed in erythema multiforme minor

A 
• Pain
     - Mild to severe
     - May --> dehydration
• Erythematous patches
     - Epithelial necrosis
• Aphthous-type ulcers
• Multiple superficial extensive irregular ulcers
• Some vesicles/bullae
     - Soon rupture
• Lips affected more than other sites.  Gingivae less affected than other sites
     - Hemorrhagic lips
136
Q

Which sites in oral lesions from erythema multiforme minor are affected?

A

Lips affected more than other sites. Gingivae less affected than other sites.

137
Q

Skin lesions observed in erythema multiforme?

A
  • Target lesions: concentric erythematous rings

* Macules, papules, vesicles, bullae, etc.

138
Q

Resolution of erythema multiforme minor

A

Usually resolves spontaneously in 2-6 weeks

139
Q

Erythema multiforme major is also known as…

A

Stevens-Johnson syndrome

140
Q

Clinical presentation of erythema multiforme major

A
  • Oral, eye, and genital lesions
  • Symblepharon may occur
  • Often drug induced
141
Q

Mortality rate for those with erythema multiforme?

A

~2-10% mortality

142
Q

Toxic epidermal necrolysis (TEN) is also known as….

A

Lyell’s disease

143
Q

Etiology of toxic epidermal necrolysis (TEN)…

A

Usually drug induced

144
Q

Clinical presentation of toxic epidermal necrolysis

A
  • Sloughing and ulceration of entire skin and mucosae

* Resembles burn victim

145
Q

How long does it take skin & oral lesions to resolve in someone with toxic epidermal necrolysis?

A

Skin lesions resolve in less than 4 weeks. Oral takes longer.

146
Q

Percent mortality rate for toxic epidermal necrolysis (TEN)

A

~34% mortality

147
Q

Histology of erythema multiforme

A 
• Necrotic keratinocytes
• Spongiosis
• Vesicles in epithelium may extend below the epithelium
• Interface infiltrate
      - Lymphocytes, neutrophils
      - +/- eosinophils
• Perivascular inflammation
148
Q

Histology is characteristic, but not pathognomonic for this disease…

A

Erythema multiforme

149
Q

Immunofluorescence for erythema multiforme is…

A

non-specific

150
Q

Describe corticosteroid treatment for erythema multiforme

A

• Corticosteroid use is controversial

 - Effectiveness is questionable
 - May be dangerous in TEN
151
Q

Management of erythema multiforme

A

• Bland mouthwashes may be adequate in erythema minor
• Eliminate triggers
- Antiviral agents in cases triggered by HSV
• Rehydrate (IV if necessary)

152
Q

Treatment for toxic epidermal necrolysis

A

• Treated in burn unit
• Intravenous pooled human Ig
- Blocks Fas ligand
- which induces epithelial cell apoptosis

153
Q

What is reactive arthritis/Reiter’s syndrome?

A

• Abnormal immune reaction to microbial antigen (STD or dysentery)

154
Q

Demography of reactive arthritis/Reiter’s syndrome?

A

Young adult white males

155
Q

Reactive arthritis/Reiter’s syndrome associated with this pehnotype…

A

HLA-B27 phenotype

156
Q

Clinical presentation of reactive arthritis/Reiter’s syndrome…

A 
• 1-4 weeks after exposure
• Acute onset of triad:
     - Non-specific urethritis
     - Conjunctivitis
     - Arthritis
• Oral in fewer than 20%
     - Ulcers (RAU)
     - Papules
     - Erythema migrans
• Skin lesions in some:
     - Histology is psoriasiform
157
Q

Management of reactive arthritis/Reiter’s syndrome

A

NSAIDs for arthritis

158
Q

Resolution of reactive arthritis/Reiter’s syndrome

A

Lasts weeks to months with recurrence

159
Q

Skin lesions that present in reactive arthritis/Reiter’s syndrome show this type of histology…

A

Histology is psoriasiform

160
Q

Timeline of acute graft v host disease and chronic graft v host disease

A
  • Acute: within first 100 days of transplant

* Chronic: continuation of acute GVHD or starts later than 100 days

161
Q

How many bone marrow recipients are affected by AGVHD and CGVHD?

A
  • Acute: affects 50% bone marrow recipients

* Chronic: in up to 60% of bone marrow recipients

162
Q

Clinical presentation of acute graft v host disease

A
  • Skin mild to severe like “TEN”

* Severe GI symptoms and liver dysfunction

163
Q

Clinical presentation of chronic graft v host disease

A
  • Mimics autoimmune diseases (SLE, Sjogren, etc.)

* Skin changes resemble lichen planus or systemic sclerosis

164
Q

In what percentage of chronic and acute graft v host disease are oral changes observed in..?

A
  • In 33 to 75% of acute GVHD

* In ~80% of chronic GVHD

165
Q

Describe oral changes observed in GVHD

A

• Lichenoid appearance:
- Histology resembles lichen planus but inflammatory response is not as intense
- With advanced cases, collagen deposition resembles systemic sclerosis
• Burning
• 2nd candidiasis
• Ulcers
• Xerostomia

166
Q

What is xerostomia?

A

Immune destruction of salivary glands

167
Q

Histology of oral changes observed in GVHD resembles…

A

lichen planus, however, the inflammatory response is not as intense

168
Q

Describe xerostomia as it relates to GVHD

A

Minor glands show periductal inflammation in early stages, with gradual acinar destruction and periductal fibrosis later

169
Q

What does direct immunofluorescence identify?

A

Identifies factors in patient TISSUE

170
Q

Describe direct immunofluorescence process

A

• Patient tissue incubated with F-labeled antibody against:

 - Tissue bound autoantibody
 - Tissue antigen (C3, fibrinogen)
 - Foreign antigen (viral antigen)
171
Q

What does indirect immunofluorescence identify?

A

Identifies autoantibody in patient SERUM

172
Q

Describe indirect immunofluorescence process

A
  • Animal mucosa incubated with patient serum
  • Autoantibody (if present) in serum attaches to corresponding structure in mucosa
  • Incubated with F-labeled antibody against autoantibody

Oral Pathology (9 decks)

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