Vesiculobullous and Ulcerative Lesions Flashcards
Oral lichen planus vs cutaneous
more frequent?
more persistent/resistant?
Oral lichen planus (OLP) more frequently than
the cutaneous LP
OLP tends to be more persistent and more
resistant to treatment
LP
age
(3)
- Occurs in fourth to eighth decades
- mean age in 5th decade
- Rare in children
LP
—% incidence; —% with oral lesions have concomitant skin
lesions)
* —% (cutaneous incidence); –% also have oral lesions
* White females (60%)
3 to 4, 25
0.5 to 1, 50
LP
of 362 cases
- Candidiasis –%
- BMS –%
- OLP –%
12
10
8
LP
distribution
Bilateral and often quasi-symmetric
distribution
LP
* Oral site frequency: (4)
* Skin sites: (4)
- buccal mucosa
- tongue
- gingiva
- lips
forearm, shin, scalp, genitalia
LP
Pathophysiology
(3)
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
LP
Etiology
(5)
● 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
LP
Etiology Instigating Factors
(2)
Co-morbidities are contributary
- Diabetes
Vices are contributary
- EtOH, tobacco, etc.
LP
Clinical Presentation
(4)
● Erythematous
● Ulcerated
● Keratotic striations
● episodic pain to severe discomfort
LP
Clinical Symptoms
(4)
● asymptomatic
● itching
● episodic pain
● severe discomfort
LP
Clinical Types
(4)
Reticular -
Erosive –
Patch –
Bullous –
Reticular -
Erosive –
Patch –
Bullous –
Reticular - most common
Erosive – most painful
Patch – simulates dysplasia
Bullous – clinically similar to diseases of greater
morbidity
Reticular Lichen Planus
(3)
● lacy
● striated
● “Wickham” striae
Erosive Lichen Planus
(4)
Buccal and labial mucosa
tongue laterodorsum
Gingiva
Palate (???)
Erosive Lichen Planus
apperance (2)
Large, irregular atrophic erythematous patches
diffuse outlines
Progress to ulcerations, pseudomembranous cover
Erosive Lichen Planus
pain
symptoms
sympoms result in
Episodic pain to severe discomfort
Symptoms may persist weeks or longer
Symptoms result in weight loss, nutritional
deficiencies and depression
LP
Differential Diagnosis
(3)
lichenoid dysplasia
contact stomatitis
lichenoid reaction
LP
Treatment Goals
There is no cure, therefore;
(3)
Reduce length and severity of symptoms
Resolve oral mucosal lesions
Reduce risk of malignant degeneration to
squamous cell carcinoma
LP
Treatment Issues
Maintain good oral hygiene
because
meticulous oral hygiene reduces
symptom severity
Oral hygiene is difficult to accomplish
during active disease
LP
Treatment
(4)
Oral anesthetic rinse (1% Dyclonine solution)
Antibiotics
Antifungals (with steroid);
nystatin with triamcinolone
(Mycostatin II)
Corticosteroids
LP
Treatment Regimens
Topicals -
4-6 week course
- most popular; best success with steroid carriers
LP
Treatment Regimens
Mild –
(2)
cortisone 5% ointment
triamcinolone 0.1% ointment
LP
Treatment Regimens
Moderate –
(3)
cortisone 10% ointment
fluocinonide gel 0.05%
dexamethasone 0.05% ointment
LP
Treatment Regimens
Potent –
(2)
clobetasol 0.05% ointment/gel
halobetasol 0.05% ointment
Steroid Carriers vs. Bleaching Trays
need to border mold the
impressions so tray extends
to mucobuccal folds
LP
Treatment
Intra-lesion steroid injections
(2)
12 mg/week dexamethasone for 8 weeks
5 -10 mg/week triamcinolone PRN
LP
Treatment
Systemic steroids
Prednisone ~
2 – 3 weeks
loading dose 0.5 to 1 mg/kg/day (40 -80mg/qd)
- Need tapering down regimen
LP
Treatment
Methotrexate (antimetabolite)
10 to 20mg once weekly for 4- 12 weeks
LP
Other treatments
Hydroxychloroquine (Plaquenil)
(2)
-disease-modifying anti-rheumatic
drug (DMARD); anti-malarial
- relieve inflammation, swelling,
stiffness, and joint pain
LP
Other treatments
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
LP
Other treatments
Calcineurin inhibitors
(3)
pimecrolimus cream
tacrolimus ointment
Psychotic side effects when used systemically
LP Untreated risks
Malignant potential Risk
– %
(2) conditions
have the greatest risk
Untreated risks
0.1 – 0.2
Erosive and ulcerative
Aphthous Stomatitis
Three Types
- Minor
- Major
- Herpetiform
Aphthous Stomatitis
Affects 18 to 27% of the population;
prevalence is approximately –%
20
Aphthous
Stomatitis
Etiology - unknown
(4)
- 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)
Aphthous
Stomatitis
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)
Aphthous
Stomatitis
Clinical Description of Ulcers
(3)
- 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
Aphthous Minor
(5)
- most common subtype
- Single but more often
multiple - Less than 1 cm in diameter
- Oval to round shape
- Healing within 7 to 14 days
Aphthous Major
(a.k.a. Sutton’s Disease)
(6)
- 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
Herpetiform Aphthous Stomatitis
(7)
- 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
Aphthous
Stomatitis
Diagnosis
(3)
- Usually has diagnostic clinical
appearance of focal, well-
defined ulcers involving non-
keratinized mucosa - History helpful; a recurrent
process - Positive family history
Aphthous
Stomatitis
Differential Diagnosis
(4)
- Traumatic ulcer
- Chancre
- Recurrent intraoral herpes
simplex (HSV-1) stomatitis - Cyclic neutropenia
Aphthous
Stomatitis
Treatment
(7)
- Symptomatic therapy may be adequate.
- Systemic causative factors, if present, should be
addressed. - Tetracycline-based oral rinses may be helpful.
- Corticosteroid therapy - most rational
- most consistently effective - Other immunomodulating drugs may be helpful
(dapsone, hydroxychloroquine, topical tacrolimus,
amelexanox). - Colchicine (0.6–1.2 mg/d) is sometimes beneficial.
- Thalidomide treatment has shown efficacy in
clinical trials
- Corticosteroid therapy - most rational
- most consistently effective
(3)
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
Recurrent Aphthous Stomatitis
(4)
- 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
Benign Mucous Membrane Pemphigoid
(BMMP)
Etiology
(2)
- Autoimmune; trigger
unknown - Autoantibodies directed
against basement
membrane zone antigens
causes ulceration
Benign Mucous Membrane Pemphigoid
(BMMP)
Clinical Presentation
- 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)
Nikolsky Sign
(2)
- 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
Benign Mucous Membrane Pemphigoid
(BMMP)
Diagnosis
(2)
- Biopsy
- Direct immunofluorescent
examination
Benign Mucous Membrane Pemphigoid
(BMMP)
Differential Diagnosis
(5)
- Pemphigus vulgaris
- Erythema multiforme
- Erosive lichen planus
- Lupus erythematosus
- Epidermolysis bullosa acquisita
Benign Mucous Membrane Pemphigoid
(BMMP)
Microscopic Findings
(3)
- Subepithelial cleft formation
- Linear pattern IgG and complement 3 (C3) along
basement membrane zone; less commonly IgA - Direct immunofluorescence examination positive in
80% of cases
Mucous Membrane Pemphigoid
Treatment
(4)
- Topical corticosteroids
- Systemic prednisone, azathioprine, or
cyclophosphamide - Tetracycline/niacinamide
- Dapsone
Mucous Membrane Pemphigoid
Prognosis
(3)
- 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)
Pemphigus Vulgaris
Etiology
(2)
- 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
Pemphigus Vulgaris
Clinical Presentation
(2)
- Over 50% of cases develop
oral lesions as the initial
manifestation - Oral lesions develop in 70% of
cases
Pemphigus Vulgaris
Clinical Presentation
(3)
- Painful, shallow irregular
ulcers with friable adjacent
mucosa - Nonkeratinized sites (buccal,
floor, ventral tongue) often are
initial sites affected - Nikolsky sign
Pemphigus Vulgaris
Microscopic Findings
(8)
- 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
Pemphigus Vulgaris
Diagnosis
(3)
- Clinical appearance
- Mucosal manifestations
- Direct/indirect
immunofluorescent studies
Pemphigus Vulgaris
Differential Diagnosis
(5)
- Mucous membrane (cicatricial)
pemphigoid - Erythema multiforme
- Erosive lichen planus
- Drug reaction
- Paraneoplastic pemphigus
Pemphigus Vulgaris
Treatment
(4)
- Systemic immunosuppression
- Prednisone, azathioprine,
mycophenolate mofetil,
cyclophosphamide - Plasmapheresis plus
immunosuppression - IV Ig for some recalcitrant cases
Pemphigus Vulgaris
Oral Management
(3)
- Periodontal disease aggravates the
condition - forming a relationship for
maintenance and observation with a
periodontist is prudent management - Restorative treatment aggravates the
condition
Pemphigus Vulgaris
Prognosis
(2)
- Guarded
- Approximately a 5% mortality rate
secondary to long-term systemic
corticosteroid–related complications
Benign Mucous Membrane Pemphigoid
Cicatrixal Pemphigoid
(3)
Eyes and genitalia are
often involved.
Blister is beneath the
epithelium.
Antibodies attack the
basement membrane
Pemphigus Vulgaris
(3)
Primarily a skin disorder.
Blister is within the
epithelium.
Antibodies attack the
intercellular bridges.
Lupus Erythematosus
Etiology
(3)
- An autoimmune-
/immunologically mediated
condition - Antibodies demonstrable
against an array of cytoplasmic
and nuclear antigens - Most often occurs in women
Lupus Erythematosus
Three forms :
- Chronic cutaneous (CCLE) or
discoid (DLE) - Subacute cutaneous (SCLE)
- Systemic (SLE)
Lupus Erythematosus
Clinical Presentation
(7)
- 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.
Lupus Erythematosus
Topical Treatments
(3)
- 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
Lupus Erythematosus
Prognosis
(3)
- 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.
Erythema
Multiforme
Etiology (4)
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.
Erythema
Multiforme
Clinical Presentation
(5)
- 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 - Predilection for young adults;
20-40 years
Erythema
Multiforme
Target or iris skin lesions may be noted over —
* (2) lesions may occur.
* Usually self-limiting; – week course
* Recurrence is common
extremities.
Genital and ocular
2- to 4-
Erythema
Multiforme
Diagnosis
(4)
Appearance (note lip crusting)
* Rapid onset
* Multiple site involvement in one-half of cases
* Biopsy results often helpful, but not always diagnostic
Erythema
Multiforme
Differential Diagnosis
(4)
- 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
Erythema Multiforme
Treatment
(4)
- 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. - See “Therapeutics” section for details
Erythema Multiforme
Prognosis
(2)
- Generally excellent
- Recurrences common
Stevens-Johnson Syndrome
Etiology
(5)
- 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”
Stevens-Johnson Syndrome
Clinical Presentation 1
(4)
- 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
Stevens-Johnson Syndrome
Clinical Presentation 2
(5)
- 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.
Stevens-Johnson Syndrome
Diagnosis
(2)
- Usually made on clinical
grounds - Histopathology is not
diagnostic.
Stevens-Johnson Syndrome
Differential Diagnosis
(5)
- Pemphigus vulgaris
- Paraneoplastic pemphigus
- Mucous membrane (cicatricial)
pemphigoid * Bullous
pemphigoid - Acute herpetic
gingivostomatitis - Stomatitis medicamentosa
Stevens-Johnson Syndrome
Treatment
(5)
- 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
Stevens-Johnson Syndrome
Prognosis
(2)
- Good; self-limiting usually
- Recurrences not uncommon
