vesiculobollous and ulcerative lesions Flashcards
1
Q
Lichen Planus general info
* Occurs in what decades
* mean age?
* Rare in what age group?
* % incidence;% with oral lesions have concomitant skin lesions
* % (cutaneous incidence); % also have oral lesions
* predominate demo?
A
- Occurs in fourth to eighth decades
- mean age in 5th decade
- Rare in children
- 3 to 4% incidence; 25% with oral lesions have concomitant skin
lesions) - 0.5 to 1% (cutaneous incidence); 50% also have oral lesions
- White females (60%)
2
Q
distribution of LP
A
- Bilateral and often quasi-symmetric
distribution - Oral site frequency:
1. buccal mucosa
2. tongue
3. gingiva
4. lips - Skin sites: forearm, shin, scalp, genitalia
3
Q
LP pathophys
A
not infectious/ not hypersensitivity
autoimmune disease; T-lymphocytes attack Langerhan cells in epithelium of affected areas
Causes chronic inflammatory lesions with varying episodes of intensity
4
Q
LP etiology
meds?
vax?
allergens?
A
● NSAID’s (ibuprofen and naproxen)
● Various medications for heart disease, hypertension (hydrochorthiazide, etc.) , rheumatoid arthritis
Hepatitis C infection and other types of liver disease
* Vaccines - Hepatitis B, various flu vaccines, effect of the COVID vaccine uncertain
* Food allergens, dental materials or other substances
5
Q
contributary factors to LP
A
Co-morbidities are contributary
- Diabetes
Vices are contributary
- EtOH, tobacco, etc
6
Q
Erosive LP clinical presentation
A
● Erythematous
● Ulcerated
● Keratotic striations
● episodic pain to severe discomfort.
7
Q
LP progression of symptoms
A
● asymptomatic
● itching
● episodic pain
● severe discomfort.
8
Q
clinical forms of LP
A
Reticular - most common
Erosive – most painful
Patch – simulates dysplasia
Bullous – clinically similar to diseases of greater morbidity
9
Q
reticular LP
A
● lacy
● striated
● “Wickham” striae
10
Q
A
reticular LP
11
Q
Erosive Lichen Planus locations
A
Buccal and labial mucosa
tongue laterodorsum
Gingiva
Palate (???)
12
Q
erosive LP presentation
A
Large, irregular atrophic erythematous patches
diffuse outlines
Progress to ulcerations, pseudomembranous cover
13
Q
Erosive LP symptoms
A
Episodic pain to severe discomfort
Symptoms may persist weeks or longer
Symptoms result in weight loss, nutritional deficiencies and depression.
14
Q
A
bullous LP
15
Q
Bullous Lichen Planus app
A
Small broken bullae/ulcers near white keratotic striae.
16
Q
Differential Diagnosis for LP
A
lichenoid dysplasia
contact stomatitis
lichenoid reaction.
17
Q
Treatment Goals LP
A
There is no cure, therefore;
Reduce length and severity of symptoms
Resolve oral mucosal lesions
Reduce risk of malignant degeneration to squamous cell carcinoma
18
Q
Treatment Issues LP
A
Maintain good oral hygiene because meticulous oral hygiene reduces symptom severity
Oral hygiene is difficult to accomplish during active disease
19
Q
Treatment options for LP (rx)
A
Oral anesthetic rinse (1% Dyclonine solution)
Antibiotics
Antifungals (with steroid); nystatin with triamcinolone
Corticosteroids
20
Q
tx regimens LP
A
gel better than cream, less soluble
21
Q
steroid carriers
A
Need to border mold the
impressions so tray extends
to mucobuccal folds for rx delivery
22
Q
Intra-lesion steroid injections for LP
A
12 mg/week dexamethasone for 8 weeks
5 -10 mg/week triamcinolone PRN
23
Q
systemic roids for LP
A
24
Q
antiviral tx for LP
A
Hydroxychloroquine (Plaquenil)
- relieve inflammation, swelling, stiffness, and joint pain
25
Thalidomide
Bad history when used in pregnancy for anxiety, morning sickness, headache, etcc. (1950s);
Thalidomide babies had lack of appendage development (arms,
legs). Other aplasias - ears or malformed kidneys.
Contemporary use for inflammatory mucocutaneous diseases
Treatment
26
Calcineurin inhibitors
used for?
forms?
what side effects when used systemically?
used for LP
pimecrolimus cream or tacrolimus ointment
Psychotic side effects when used systemically
27
untx risks of LP
Malignant potential Risk
– 0.1 – 0.2%
Erosive and ulcerative conditions have the greatest risk
28
Aphthous Stomatitis types
1. Minor
2. Major
3. Herpetiform
29
Aphthous Stomatitis etiology
* viral or other infectious agent?
* probably represents?
* Triggers include?
* Human leukocyte antigen (HLA) subtype susceptibility?
* no viral or other infectious agent identified
* probably represents focal immunodysfunction but the specific mechanism is undetermined.
* Triggers include increased stress/anxiety, hormonal changes, dietary factors, trauma, etc...)
* Human leukocyte antigen (HLA) subtype susceptibility a factor in some cases (-B12, -B51, and others)
30
aphthous stomatitis membrane alterations
Alterations in mucosal membrane barrier permeability may be a factor because of co-morbidity associations with:
HIV/AIDS
bone marrow suppression
Neutropenia
gluten sensitivity
Crohn’s disease
ulcerative colitis
food allergy
Behçet disease
dietary deficiencies: iron, Zn, vitamin B12 (folate)
31
Aphthous
Stomatitis clinical description of ulcer
Recurrent, self-limiting, painful ulcers
* Usually restricted to nonkeratinized oral and pharyngeal mucosa (not hard palate or attached gingiva)
* Well-demarcated ulcers with yellow fibrinous base and erythematous halo
32
aphthous stomatitis
33
Aphthous Minor:
commonality?
* number?
* size?
* shape?
* healing time?
most common subtype
* Single but more often multiple
* Less than 1 cm in diameter
* Oval to round shape
* Healing within 7 to 14 days
34
aphthous minor
35
aphthous major:
* size?
* number?
* depth?
*edges?
* length?
* Often heal with?
* 1 cm or greater in diameter
* Single or less commonly several
* Deep
* To ragged edges with elevated edematous margin
* May persist for several weeks to months
* Often heal with scarring
36
aphthous major
37
Herpetiform Aphthous Stomatitis
* least common variant
* Grouped superficial ulcers 1-2 mm dia
* crops of 10 to 100 lesions
* lesions coalesce
* In nonkeratinized and keratinized tissues
* Healing within 7 to 14 days
* No etiologic role for herpes simplex virus
38
herpetiform aphthous stomatitis
39
Aphthous Stomatitis diagnosis
* Usually has diagnostic?
* History?
* family history?
* Usually has diagnostic clinical appearance of focal, well- defined ulcers involving non- keratinized mucosa
* History helpful; a recurrentprocess
* Positive family history
40
aphthous stomatitis dif dx
* Traumatic ulcer
* Chancre
* Recurrent intraoral herpes simplex (HSV-1) stomatitis
* Cyclic neutropenia
41
Aphthous Stomatitis - Treatment
1. what may be adequate?
2. what, if present, should be addressed?
3. what oral rinses may be helpful?
4. what is most rational- most consistently effective? forms of this?
5. Other helpful agents?
6. Colchicine?
7. Thalidomide?
1. Symptomatic therapy may be adequate.
2. Systemic causative factors, if present, should be addressed.
3. Tetracycline-based oral rinses may be helpful.
4. Corticosteroid therapy - most rational- most consistently effective
a. Topical corticosteroids as gels, creams, or ointment 4 to 6 times/d to early lesions
b. Intralesional corticosteroid injections
c. Short-duration systemic corticosteroids (low to moderate doses
5. Other immunomodulating drugs may be helpful (dapsone, hydroxychloroquine, topical tacrolimus, amelexanox).
6. Colchicine (0.6–1.2 mg/d) is sometimes beneficial.
7. Thalidomide treatment has shown efficacy in clinical trials
42
Recurrent Aphthous Stomatitis
Recur as? more common recurrent form?
* In AIDS patients, lesions are typically?
* Recur as minor or major variants
Minor is more common recurrent form
* Minor; episodic: 1– 4 episodes/year;
few lesions, usually minor or herpetiform)
* Major; almost continuous ulcerations; disabling, large, or severe lesions)
* In AIDS patients, lesions are typically more severe and may occur on any oral surface
43
Benign Mucous Membrane Pemphigoid (BMMP)
Etiology
* Autoimmune; trigger unknown
* Autoantibodies directed against basement membrane zone antigens causes ulceration
44
Benign Mucous Membrane Pemphigoid (BMMP)
Clinical Presentation
* intraoral sites?
* Usually in what age?
* gender?
* Ocular lesions noted in how many cases?
* scarring tendency?
* Vesicles and bullae followed by ulceration
* Multiple intraoral sites (occasionally gingiva only)
* Usually in older adults
* 2:1 female predilection
* Ocular lesions noted in one-third of cases
* scarring tendency in ocular, laryngeal, nasopharyngeal, and oropharyngeal tissues (Cicatricial Pemphigoid)
45
Benign mucus membrane pemphigoid
46
Nikolsky Sign
- Epithelial splitting
- application of firm lateral shearing force on uninvolved skin or mucosa can produce a surface slough or induce vesicle formation
- Broken bullae leave open, painful ulcerations
47
Benign Mucous Membrane Pemphigoid
(BMMP)
Diagnosis
* Biopsy
* Direct immunofluorescent
examination
48
BMMP dif dx
* Pemphigus vulgaris
* Erythema multiforme
* Erosive lichen planus
* Lupus erythematosus
* Epidermolysis bullosa acquisita
49
BMMP microscope
* Subepithelial cleft formation
* Linear pattern IgG and complement C3 along basement membrane zone; less commonly IgA
* Direct immunofluorescence examination positive in 80% of cases
50
Mucous Membrane Pemphigoid Treatment
* Topical?
* Systemic?
* Abx?
* Topical corticosteroids
* Systemic prednisone, azathioprine, or cyclophosphamide
* Tetracycline/niacinamide
* Dapsone
51
MMP prognosis?
Morbidity related to?
* Management often difficult due to?
* Management often requires?
Morbidity related to mucosal scarring (oropharyngeal, nasopharyngeal, laryngeal, ocular, genital)
* Management often difficult due to variable response to corticosteroids
* Management often requires multiple specialists working in concert (dental, dermatology, ophthalmology, otolaryngology)
52
Benign Mucous Membrane Pemphigoid
Cicatrixal Pemphigoid
53
Cicatrixal Pemphigoids?
Recently ruptured bulla with epithelial cover still attached, likley to form scar in ocular, laryngeal, nasopharyngeal, and oropharyngeal tissues (Cicatricial Pemphigoid)
54
# is this symptomatic?
pt with BMMP
Massive, relatively painless sloughing of buccal mucosa, Cicatricial Pemphigoid
55
Benign Mucous Membrane Pemphigoid
Gingival Variant
can also be localized to the gingiva
56
other areas affected by BMMP
Eye, Mouth, Genitalia, Sometimes Skin
57
Pemphigus Vulgaris
Etiologies
* An autoimmune disease where antibodies are directed toward the desmosome-related proteins of the epithelial intercellular bridges (desmoglein 3 or desmoglein 1)
* A drug-induced form exists with less specificity in terms of immunologic features, clinical presentation, and histopathology
58
Pemphigus Vulgaris:
* Over % of cases develop oral lesions as the initial
manifestation?
* Oral lesions develop in % of cases?
* Over 50% of cases develop oral lesions as the initial
manifestation
* Oral lesions develop in 70% of cases
59
# app? initial sites? sign?
Pemphigus Vulgaris Clinical Presentation
* Painful, shallow irregular ulcers with friable adjacent mucosa
* Nonkeratinized sites (buccal, floor, ventral tongue) often are initial sites affected
* + Nikolsky sign
60
Pemphigus Vulgaris Microscopic Findings
Separation or clefting of suprabasal from basal layer of epithelium
* Intact basal layer of surface epithelium
* Vesicle forms at site of epithelial split
* Direct immunofluorescence examination positive in all cases
*** IgG** localization to intercellular spaces of epithelium
* **C3 **localization to intercellular spaces in **80%** of cases
* **IgA** localization to intercellular spaces in 30% of cases
* General correlation with severity of clinical disease
61
Pemphigus Vulgaris
Diagnosis accomplished with?
* Clinical appearance
* Mucosal manifestations
* Direct/indirect immunofluorescent studies
62
PV dif dx
* Mucous membrane (cicatricial)
pemphigoid
* Erythema multiforme
* Erosive lichen planus
* Drug reaction
* Paraneoplastic pemphigus
63
Pemphigus Vulgaris Treatment
* Systemic plan?
* options for this?
* Plasmapheresis?
* some recalcitrant cases may need?
* Systemic immunosuppression
* Prednisone, azathioprine, mycophenolate mofetil,
cyclophosphamide
* Plasmapheresis plus immunosuppression
* IV Ig for some recalcitrant cases
64
# perio dx? restorative tx?
Pemphigus Vulgaris
Oral Management
* Periodontal disease aggravates the condition
* forming a relationship for maintenance and observation with a periodontist is prudent management
* Restorative treatment aggravates the condition
65
# what can mortality be related to?
Pemphigus Vulgaris
Prognosis
* Guarded
* Approximately a 5% mortality rate secondary to long-term systemic corticosteroid–related complication
66
Pemphigus Vulgaris Ab's
Autoantibodies Against Intracellular Bridges
67
Lupus Erythematosus Etiology
* An autoimmune/immunologically mediated condition
* Antibodies demonstrable against an array of cytoplasmic and nuclear antigens
* Most often occurs in women
68
Lupus Erythematosus
Three forms :
1. Chronic cutaneous (CCLE) or
discoid (DLE)
2. Subacute cutaneous (SCLE)
3. Systemic (SLE)
69
Lupus Erythematosus Clinical Presentation
* demo with highest incidence
* Predominates in women >?
* 80% of patients have what other finding?
* % of SLE patients with oral mucosal findings?
* Oral mucosal lesions may appear as?
* Labial vermilion?
* Oral findings are most common in which forms?
* Black females have highest incidence
* Predominates in women > 40 years
* 80% of patients have concurrent cutaneous findings
* 30 to 40% of SLE patients have oral mucosal findings
* Oral mucosal lesions may appear lichenoid, keratotic, and erosive.
* Labial vermilion with crusted, exfoliative, erythematous, and keratotic appearance
* Oral findings are most common in CCLE or DLE.
70
lupus erythematosus
71
pt also has malar rash
lupus erythematosus
72
Lupus Erythematosus
Topical Treatments
* Fluocinonide gel/cream 0.05% 60 g; apply after meals and at bedtime
* Tacrolimus (Protopic) ointment 0.1% 30 g; apply after meals 3 times daily, do not eat or drink for 30 min
* Intralesional therapy: triamcinolone acetonide 5– 10 mg/mL; inject 1–3 mL per session with sessions at 3–4 wk intervals
73
# dif forms?
Lupus Erythematosus
Prognosis
* Good prognosis with CCLE or DLE form
* Variable prognosis with SLE
* SCLE has an intermediate prognosis between that of SLE and CCLE or DLE forms.
74
Erythema Multiforme Etiologies:
* mediated by reactive process? possibly related to?
* * Many cases preceded by infection with?; less often with?
* * drugs?
* * Another trigger may be what therapy?
* Immunologically mediated reactive process, possibly related to circulating immune complexes
* * Many cases preceded by infection with herpes simplex; less often with Mycoplasma pneumoniae or other organisms
* * May be related to drug consumption, including sulfonamides, other antibiotics, analgesics, phenolphthalein-containing laxatives, barbiturates
* * Another trigger may be radiation therapy.
75
Erythema Multiforme Clinical Presentation
* onset/app?
* Early mucosal lesions are?
* May affect what surfaces? how? most commonly affected region and app?
severe form known as?
* Acute onset of multiple, painful, shallow ulcers and erosions with irregular margins
* Early mucosal lesions are macular, erythematous, and occasionally bullous.
* May affect oral mucosa and skin synchronously or metachronously
Lips most commonly affected with eroded, crusted, and hemorrhagic lesions (serosanguinous exudate)
known as Stevens-Johnson syndrome when severe
76
acute onset, what is likely?
erythema multiforme
77
EM demo
Predilection for young adults;
20-40 years
78
erythema multiforme
79
Erythema Multiforme systemic Clinical Presentation:
* lesions noted over extremities?
* Genital and ocular lesions?
* time frame?
* Recurrence?
* Target or iris skin lesions may be noted over extremities.
* Genital and ocular lesions may occur.
* Usually self-limiting; 2- to 4-week course
* Recurrence is commo
80
Erythema Multiforme Diagnosis:
* Appearance?
* onset?
* how many sites involved?
* Biopsy?
* Appearance (note lip crusting)
* Rapid onset
* Multiple site involvement in one-half of cases
* Biopsy results often helpful, but not always diagnostic
81
EM dif dx
* Viral infection, in particular, acute herpetic gingivostomatitis (Note: Erythema multiforme rarely
affects the gingiva.)
* Pemphigus vulgaris
* Major aphthous ulcers
* Erosive lichen planus
* Mucous membrane (cicatricial) pemphigoid
82
Erythema Multiforme Treatment:
* Mild (minor) form:
* * Severe (major) form:
* * If evidence of an antecedent viral infection or trigger exists, what can help?
* Mild (minor) form: symptomatic/supportive treatment with adequate hydration, liquid diet, analgesics, topical corticosteroid agents
* * Severe (major) form: systemic corticosteroids, parenteral fluid replacement, antipyretics
* * If evidence of an antecedent viral infection or trigger exists, systemic antiviral drugs during the disease or as a prophylactic measure may help.
83
EM prognosis
* Generally excellent
* Recurrences common
84
Stevens-Johnson Syndrome Etiology:
* A complex mucocutaneous disease
affecting?
* Most common trigger:
* Infection with what other spp can also may
serve as a trigger?
* Medication trigger?
* Sometimes referred to as ?
* A complex mucocutaneous disease affecting two or more mucosal sites simultaneously
* Most common trigger: antecedent recurrent herpes simplex infection
* Infection with Mycoplasma also may serve as a trigger.
* Medications may serve as initiators in some cases.
* Sometimes referred to as “erythema multiforme major”
85
Stevens-Johnson Syndrome Clinical Presentation 1
* areas usually affected initially?
* lesion progression?
* later appears?
* Pseudomembrane involved?
* Labial vermilion and anterior portion of oral cavity usually affected initially
* macular lesions erode, then slough, and ulcerate
* later appear crusted and hemorrhagic.
* Pseudomembrane; foul-smelling presentation as bacterial colonization supervenes
86
Stevens-Johnson Syndrome Clinical Presentation 2:
* Posterior oral cavity and oropharyngeal involvement leads to?
* Eyes?
* Genitals?
* Cutaneous involvement?
* lesions characteristic on skin?
* Posterior oral cavity and oropharyngeal involvement leads to odynophagia, sialorrhea, drooling
* Eye (conjunctival) involvement may occur.
* Genital involvement may occur.
* Cutaneous involvement may become bullous.
* Iris or target lesions are characteristic on skin
87
Stevens-Johnson Syndrome
Diagnosis
* Usually made on?
* Histopathology?
* Usually made on clinical grounds
* Histopathology is not diagnostic
88
SJS dif dx
* Pemphigus vulgaris
* Paraneoplastic pemphigus
* Mucous membrane (cicatricial)
pemphigoid
* Bullous pemphigoid
* Acute herpetic gingivostomatitis
* Stomatitis medicamentosa
89
Stevens-Johnson Syndrome
Treatment:
* symptomatic measures?
* oral rinses?
* use of what is controversial?
* Recurrent, virally associated cases may be reduced in frequency with use of?
* May require admission to?
* Hydration and local symptomatic measures
* Topical compounded oral rinses
* Systemic corticosteroid use controversial
* Recurrent, virally associated cases may be reduced in frequency with use of daily, low-dose antiviral prophylactic therapy (acyclovir, famciclovir, valacyclovir).
* May require admission to hospital burn unit
90
SJS prognosis
* Good; self-limiting usually
* Recurrences not uncommon
