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Condition Clinical Image Description
Developmental Defects of Teeth
Amelogenesis imperfecta ● Developmental disturbance that causes
abnormal enamel formation
● Teeth can appear yellow-brown or
opaque white
● Three broad phenotypes: hypoplastic,
hypomaturation, hypocalcified
Dentinogenesis imperfecta ● Developmental disturbance that causes
abnormal dentin formation
● Teeth can appear discolored (blue-gray
or yellow-brown), translucent, highly worn,
and easily fracture
● Crowns appear bulbous with cervical
constriction and obliterated pulp chamber
Dentin dysplasia ● Developmental disturbance that causes
abnormal dentin and pulpal
morphology
Dental Anomalies
Regional odontodysplasia ● Non-hereditary abnormality of dental hard
tissues in a localized region or quadrant
● Poor mineralization of dentin and
enamel (“ghost teeth”)
Key Facts
● Hypoplastic type: unusual amount or appearance of enamel
(wrinkly or smooth), but normal enamel hardness
● Hypomaturation type: defect in the maturation of enamel
crystalline structure, resulting in softer than normal enamel
that is mottled or opaque
● Hypocalcified type: defect in the mineralization of enamel,
resulting in softer enamel that is lost by attrition or abrasion
● Type I: osteogenesis imperfecta (brittle bones and blue
sclera) with opalescent teeth
● Type II: most common, affects only dentin with no systemic
effects
● Type III: very rare, brandywine type
● Type I (radicular): short roots or “rootless teeth”,
obliterated pulp chamber or chevron-shaped remnant,
affects primary and permanent dentitions
● Type II (coronal): normal roots, enlarged or thistle
tube-shaped pulp chambers, pulp stones, affects only
primary dentition
● Short roots, open apices, and enlarged pulp chambers
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Condition Clinical Image Description Key Facts

Anomalies

Congenital syphilis ● Hutchinson’s incisor: ● Occurs when a pregnant mother transmits syphilis to her
(Hutchinson’s incisor, “screwdriver-shaped” or triangular incisor child in utero or at birth
mulberry molar) ● Mulberry molar: first molars that appear
like a mulberry fruit, with extra and
rounded enamel cusps

Concresence ● Union of adjacent teeth at the level of ● Enamel, dentin, and pulp are not involved
cementum ● Occurs most frequently in maxillary molars

Gemination ● Attempt of one tooth bud to become two ● Single root and pulp chamber
separate teeth ● Occurs most frequently in incisors
● Normal number of teeth

Fusion ● Fusion of two teeth at the dentin and pulp ● Two separate pulp chambers
● One fewer tooh (# teeth - 1) ● Occurs most frequently in mandibular lateral incisors and
canines
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Condition Clinical Image Description Key Facts

Dental Anomalies

Dens invaginatus (dens in ● Invagination or infolding of the tooth ● Occurs most frequently in maxillary lateral incisors
dente) surface prior to calcification ● Communication directly to the pulp may result in pulpal
● “Tooth within a tooth” necrosis and apical periodontitis

Dens evaginatus ● Outward folding of the tooth surface ● Occurs most frequently in mandibular premolars
prior to calcifications ● Pulp horns extending into enamel may result in pulpal
● Forms a small, extra cusp necrosis and apical periodontitis

Taurodontism ● Elongation of the pulp chamber due to ● Mostly occurs in permanent molars
apical migration of the furcation ● Can occur in patients with amelogenesis imperfecta and
Down syndrome

Variations in size ● Macrodontia: a tooth or group of teeth ● Macrodontia can result from gemination or fusion
are abnormally larger ● Most common microdonts are the maxillary lateral incisors
● Microdontia: a tooth or group of teeth are (peg laterals)
abnormally smaller
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Condition Clinical Image Description Key Facts

Dental Anomalies

Variations in number ● Hyperdontia: presence of supernumerary ● Most frequent congenitally missing permanent teeth:
teeth maxillary lateral incisors, mandibular second premolars,
● Mesiodens: supernumerary tooth and third molars
between two central incisors
● Hypodontia: 1 to 6 missing teeth,
excluding third molars
● Oligodontia: greater than 6 teeth missing
● Anodontia: all teeth missing

Enamel pearl ● Small, round nodule of ectopic enamel ● Occurs most frequently at the furcation point between
most commonly on the root surface maxillary molar roots

Turner’s hypoplasia ● Trauma or periapical infection of a ● Tooth can appear white or yellow-brown
primary tooth damages ameloblasts of
developing permanent tooth
● Results in enamel hypoplasia of a single
permanent tooth

Inherited Diseases of the Orofacial Complex

Cherubism ● Autosomal dominant condition where
osteoclast overactivity results in
compensatory osteoblast activity
● Enlarged cheeks, and in more severe
cases the eyes may push upward
● Bilateral, multilocular radiolucent lesions on maxilla and
ramus, resulting in premature exfoliation of teeth
● Mandible and maxilla slowly enlarge in childhood
● After puberty, majority of cases will begin to reverse as bone
replaces cystic tissue
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Condition Clinical Image Description
Inherited Diseases of the Orofacial Complex
Cleidocranial dysplasia ● Autosomal dominant condition that
causes delayed closure of frontal
sutures and impaired osteoblast
differentiation
Inherited Diseases of the Orofacial Complex
Crouzon syndrome ● Autosomal dominant condition that
involves craniosynostosis (early closure
of cranial sutures)
Down syndrome (Trisomy 21) ● Genetic condition caused by
nondisjunction, resulting in an extra copy
of chromosome 21
● Flattened facial profile, small ears, short
neck, atrioventricular septal defect,
prominent epicanthal (upper eyelid) folds
Ectodermal dysplasia ● Group of inherited conditions of
ectodermal tissue
● 2 or more tissues of ectodermal origin are
affected (skin, sweat glands, hair, nails,
teeth, and/or mucous membrane)
Gardner syndrome ● Autosomal dominant condition that results
in multiple polyps of the colon, skin, and
skeleton and osteomas in the jaws
Key Facts
● Presents with hypoplastic or absent clavicles, prominent
skull, mandibular prognathism, and hypertelorism (wide-set
eyes)
● Associated with supernumerary teeth, prolonged retention
of primary teeth, and cleft palate
● Presents with brachycephaly (short skull), midface
deficiency or hypoplasia, frontal bossing, hypertelorism,
proptosis/exophthalmos, and intellectual disabilities
● Associated with supernumerary teeth
● Most common chromosomal abnormality
● Associated with maxillary hypoplasia, macroglossia,
decreased muscle tone, and a higher incidence of class III
malocclusion
● Delayed tooth eruption, hypodontia, supernumerary teeth,
and increased risk of periodontal disease
● Protypical triad: hypohidrosis (diminished secretions),
hypotrichosis (diminished sweating), and hypodontia
(fewer teeth)
● Associated with fine, sparse hair, wrinkly skin, and
conical-shaped teeth
● Cotton-wool appearance of the jaws
● Epidermoid cysts of the skin, supernumerary teeth, impacted
teeth, and connective tissue growths
● Multiple colonic polyps associated with malignant
transformation rate

Condition
Gorlin syndrome (nevoid basal
cell carcinoma syndrome)
Papillon-Lefevre syndrome
McCune-Albright syndrome
Neurofibromatosis type 1 (Von
Recklinghausen’ disease)
Sturge-Weber syndrome
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Clinical Image Description
Inherited Diseases of the Orofacial Complex
● Results in multiple basal cell carcinomas
and odontogenic keratocysts
● Subtype of palmoplantar keratoderma
that affects the integrity of junctional
epithelium and results in abnormal
inflammatory response
Inherited Diseases with Pigmented Changes
● Genetic condition classified by the
prototypical triad: fibrous dysplasia of
bone, café-au-lait macules, and
endocrine abnormalities
● Autosomal dominant condition causing
benign neoplasms of Schwann cells and
fibroblasts (neurofibromas)
● Café-au-lait macules and Lisch nodules
on the eyes
● Congenital condition classified by
vascular proliferations
(lymphangiomas) along the distribution
of the trigeminal nerve
Key Facts
● Odontogenic keratocysts in the presence of Gorlin syndrome
are classified as keratocystic odontogenic tumors
● Associated with macrocephaly, cleft lip/palate, and other
systemic anomalies
● Hyperkeratosis of palmar and plantar surfaces
● Associated with advanced, accelerated periodontal
disease, resulting in early loss of both the primary and
permanent dentitions
● Endocrine abnormalities include precocious (early)
puberty, hyperthyroidism, and hyperparathyroidism
● Axillary freckling (Crowe’s sign), enlarged mandibular
foramina, central nervous system tumors, seizures
● Most serious complication can be neurofibrosarcoma, a
malignant transformation of neurofibromas
● Characteristic unilateral red/purple lesions (port wine
stains)
● May present with intellectual disabilities and seizures
● Associated with supernumerary teeth and lymphangiomas
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Condition Clinical Image Description
Craniofacial Anomalies
Cleft lip ● Failure of fusion of medial nasal
prominence and maxillary prominence
Cleft palate ● Failure of fusion of the palatal shelves
Oral Manifestations of Systemic Disease
Pierre Robin sequence ● Autosomal dominant condition that
causes the prototypical triad of
micrognathia, glossoptosis, and airway
obstruction
Hemophilia ● Inherited sex-linked bleeding condition,
causing blood to not easily clot due to
reduced clotting factors
● Presents with frequent nosebleeds and
bruising that result with minor trauma
Key Facts
● Most often unilateral and left-sided
● More common in males (with or without cleft palate)
● Maternal risks during pregnancy: alcohol, tobacco,
anticonvulsants
● Surgical repair at 3-6 months old
● More common in females
● Submucous cleft palate: muscles of soft palate do not fuse,
mucous membranes are intact, often associated with bifid
uvula
● Surgical repair at 6-12 months old, feeding assistance,
speech therapy, alveolar bone grafting at 6-10 years old
and/or orthodontic treatment
● Initial mandibular hypoplasia (micrognathia) causes posterior
tongue displacement (glossoptosis) which then leads to
airway obstruction
● Associated with cleft palate, hearing loss, ear infections, and
natal teeth
● Hemophilia A: Deficiency of factor VIII
● Hemophilia B: Deficiency of factor IX
● Hemophilia C: Deficiency of factor XI
● Primarily affects males

Condition
Hodgkin’s lymphoma
Langerhans cell histiocytosis
Multiple myeloma
Paget’s disease (osteitis
deformans)
Sickle cell anemia
Von Willebrand’s disease
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Clinical Image Description
Oral Manifestations of Systemic Disease
● Malignant tumor that affects
lymphocytes and typically manifests in
the lymph nodes
● Disease of bone marrow-derived
Langerhans cells that proliferate and
build up in the organs and skeletal system
● Occurs most commonly in children and
adolescents
● Malignant tumor that affects plasma
cells, a type of white blood cell that
normally produces antibodies
● Results in multiple "punched out"
radiolucencies
● Chronic bone disease that causes
disruption in bone remodeling, where
osteoclast overactivity results in
compensatory osteoblast activity
● Structurally disorganized mosaic of bone
● Results in “cotton wool” appearance of
the skull
● Autosomal recessive condition that
causes abnormal hemoglobin
synthesis with reduced oxygen levels
● Most common in Black Americans
- ● Autosomal dominant condition caused by
deficiency of Von Willebrand factor
Key Facts
● Characterized by the presence of Reed-Sternberg cells
histopathologically
● Typically causes a painless, firm enlargement of the lymph
nodes, fevers, malaise, and anemia
● Lesions in the jaw with a characteristic "ice cream scoop"
presentation, resulting in "floating teeth"
● Localized pain and swelling of the oral cavity and severe
periodontal disease
● Elevated alkaline phosphatase levels
● CRAB: Calcium elevation (hypercalcemia), Renal failure,
Anemia, and Bone damage
● Associated with amyloidosis, the production of abnormal
antibody light chains or amyloid deposits that tend to build up
in various organs
● IV bisphosphonates and corticosteroids, such as
dexamethasone and prednisone are important treatment
modalities
● Results in dense, fragile bone that is at risk for pathologic
fracture or osteomyelitis
● Due to skull deformities, hats and dentures may no longer fit
● IV bisphosphonates are often prescribed
● Elevated alkaline phosphatase levels
● Causes moon-shaped red blood cells, swelling of
extremities, and episodes of pain during vaso-occlusive
crises
● Hair-on-end appearance of the skull, interproximal alveolar
bone step ladder patterns
● May experience tonsillitis and adenotonsillar hypertrophy
which can lead to airway obstruction and obstructive sleep
apnea
● Results in defective platelet adhesion to blood vessel wall
and decreased factor VIII
● Excessive bruising, bleeding, and nosebleeds
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Condition Clinical Image Description Key Facts

Neoplasms

Ameloblastoma ● Benign, odontogenic neoplasm ● Unilocular or multilocular radiolucency of the posterior

● Locally destructive and infiltrative, with the
potential to recur
● Behaves similarly to a malignant
neoplasm, but is painless and
slow-growing
mandible
● Bony-hard swelling, associated teeth are vital
● 3rd and 7th decade of life

Basal cell carcinoma
● Malignant neoplasm ● Older individuals, fair skin and history of sun exposure
● Can initially appear as a “pearly” nodule ● Located above the commissure lines on the face
that is flesh-colored
● Can later appear dark, shiny pink, or red
and slightly scaly

Lymphangioma ● Benign neoplasm of lymphatic vessels ● Oral lesions most commonly present on the anterior
two-thirds of the tongue as pebbly surfaces with
numerous translucent vesicles
● Associated with Sturge-Weber syndrome

Melanoma ● Malignant neoplasm of melanocytes ● When in the oral cavity, presents as a black-brown patch
and may be associated with alcohol or tobacco use
● When on the skin, associated with actinic damage or
keratosis, which are scaly patches on the skin caused by
sun exposure
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Condition Clinical Image Description Key Facts

Neoplasms

Mucoepidermoid carcinoma ● Most common salivary gland ● Can occur in children, but not exclusively
malignancy ● Most commonly affects the postero-lateral palate
● Majority are painless, slow-growing,
ulcerated mass

Odontoma ● Benign, odontogenic neoplasm ● Compound odontoma: composed of multiple tooth-like

● Painless, incidental finding associated structures
with unerupted teeth ● Complex odontoma: mass of enamel and dentin, does not
resemble a tooth

Pleomorphic adenoma ● Benign neoplasm of salivary glands ● Most commonly affects the parotid and postero-lateral
● Painless, slow-growing palate

Squamous cell carcinoma ● Malignant neoplasm ● Oral squamous cell carcinoma: lateral and ventral tongue,
● Non-wipeable white/red mass on a floor of the mouth, and anterior soft palate
cancer-prone location with surface ● 45+ years old, smoking or alcohol history
ulceration or nodularity
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Condition Clinical Image Description
Viral Infections
Human herpes virus ● HHV-1,2: herpes simplex virus (primary
herpetic gingivostomatitis, reactivates as
herpes labialis or intraoral herpetic ulcers)
● HHV-3: varicella zoster virus (chicken
pox, reactivates as shingles)
● HHV-4: Epstein-Barr virus (oral hairy
leukoplakia, mononucleosis)
● HHV-5: cytomegalovirus (CNS
involvement, mononucleosis)
● HHV-8: Kaposi sarcoma (HIV/AIDS)
Human papilloma virus ● Oncogenic virus that most often causes
benign tumors but can cause
● HPV 2, 4, 6, 11: benign
● HPV 16, 18: malignant
Key Facts
● Primary herpes simplex virus manifests in the oral cavity as
primary herpetic gingivostomatitis: pain, fever, and
clustered vesiculo-ulcerative lesions on both keratinized
and unkeratinized tissue
● Reactivated herpes simplex manifests in the oral cavity as
herpes labialis or intraoral herpetic ulcers: clustered
vesiculoulcerative lesions only on keratinized surfaces
● Primary varicella zoster virus manifests as chicken pox:
fever, pruritus, widespread maculopapular rash
● Reactivated varicella zoster virus manifests along the
dermatome and becomes latent in as shingles: pain,
vesiculoulcerative lesions, neuralgia
● Epstein-Barr virus manifests as oral hairy leukoplakia:
white, non-wipeable lesions of the lateral tongue, associated
with HIV/AIDS
● Epstein-Barr virus can also manifest as mononucleosis, a
contagious virus of teens and young adults
● Most individuals infected with cytomegalovirus are
asymptomatic, but immunocompromised hosts can present
with seizures, hearing loss, and jaundice
● Cytomegalovirus can also manifest as mononucleosis, a
contagious virus of teens and young adults
● Kaposi sarcoma is a rare complication of HIV/AIDS and
manifests as red, blue or purple lesions on the mouth,
palate, gingiva, or skin
● Exophytic warts with a cauliflower-like appearance
● Malignant strains are associated with proliferative verrucous
leukoplakia, oropharyngeal cancer, and cervical cancer
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Condition Clinical Image Description
Cysts
Dentigerous cyst ● Odontogenic, developmental cyst
● Associated with the crown of an
unerupted/impacted tooth
Lateral periodontal cyst ● Odontogenic, developmental cyst
● Pear-shaped radiolucency between roots
● Painless, incidental finding
Nasopalatine duct cyst ● Non-odontogenic, developmental cyst of
the incisive canal
● Heart-shaped radiolucency with a
sclerotic border
● Painless, incidental finding
Odontogenic keratocyst ● Odontogenic, developmental cyst
● Locally destructive and infiltrative, with
the potential to recur
● Painless and slow-growing
Key Facts
● Most common odontogenic, developmental cyst
● Unilocular radiolucency of the posterior mandible
● Typically seen from CEJ to CEJ
● Associated teeth are vital
● Unilocular or multilocular radiolucency
● Anterior hard palate posterior to teeth 8 and 9
● Associated teeth are vital
● Unilocular or multilocular radiolucency of the posterior
mandible, may be associated with unerupted teeth
● Odontogenic keratocysts in the presence of Gorlin
syndrome are classified as keratocystic odonotogenic
tumors
● Lumen contains keratinaceous debris
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Condition Clinical Image Description
Cysts
Radicular cyst ● Odontogenic, inflammatory cyst
● Also referred to as periapical cyst or
apical periodontitis
● Generally painless, unless secondarily
infected or acutely exacerbated
● May or may not present with swelling
Fibro-osseous Diseases
Cemento-osseous dysplasia ● Benign, non-neoplastic fibro-osseous
lesions of the jaw that present as mixed
radiolucency-radioopacities
● Periapical cemento-osseous dysplasia
(periapical)
● Focal cemento-osseous dysplasia
(posterior mandible)
● Florid cemento-osseous dysplasia
(widespread)
Key Facts
● Most common odontogenic (non-developmental) cyst
● Associated teeth are non-vital
● Apex or lateral radiolucency
● Root canal treatment or extraction
● Periapical cemento-osseous dysplasia is confined to the
anterior mandible
● Most often an adult female, usually African American
● Associated teeth are vital
● Usually a painless, incidental finding
● Focal cemento-osseous dysplasia is usually in the posterior
mandible
● Often in the area of prior or persistent inflammatory/infectious
foci (apical, edentulous areas); can represent chronic
osteomyelitis
● Florid cemento-osseous dysplasia involves multiple
quadrants and both arches
● Usually asymptomatic, but secondary infection is possible
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Condition Clinical Image Description Key Facts

Fibrous dysplasia ● Genetic, developmental condition ● Associated with McCune-Albright syndrome

● Normal bone marrow is replaced by ● Usually involves maxilla and >1 skeletal site
fibrous connective tissue and bone ● Diffuse, blending borders with “ground glass” appearance
and/or cementum-like tissue
● Bony hard, painless swellings

Immune-mediated Conditions

Lichen planus ● Immune-mediated mucocutaneous ● Lacy, reticulations

disease that causes basal cell cytolysis ● Multifocal, can occur cutaneously (wrists, ankles, thighs,
● Bilateral white, non-wipeable, “Wickham trunk) and orally (buccal mucosa, gingiva)
striae” of the buccal mucosa and ● Symptomatic management includes dexamethasone rinse
gingiva

Mucous membrane ● Immune-mediated mucocutaneous ● Requires referral to ophthalmology for conjunctiva

pemphigoid disease that causes hemidesmosome involvement
destruction ● Symptomatic management includes dexamethasone rinse
● Erythematous, sloughing gingiva and
mucosa
● May be painful or painless

Pemphigus vulgaris ● Immune-mediated mucocutaneous ● Requires referral to dermatology for cutaneous involvement
disease that causes desmosome ● Associated with positive Nikolsky sign
destruction ● Symptomatic management includes dexamethasone rinse
● Painful, bleeding, gingiva and mucosa

Condition
Aphthous ulcer
Central giant cell granuloma
Epulis fissuratum
Inflammatory papillary
hyperplasia
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Clinical Image Description
● Common, painful, “canker sore”
● Yellow-white, round ulceration with a
removable fibrinopurulent membrane and
an erythematous halo
● Outbreaks may be precipitated by stress
Reactive Processes
● Benign, reactive intraosseous mass of
granulation tissue that contains
multinucleated giant cells that resemble
osteoclasts (destroy bone)
● Painless, bony-hard swellings
● Fibrous hyperplasia that develops due to
the flanges of ill-fitting dentures
● Denture-associated pathology
● Papillary hyperplasia that develops on the
hard palate beneath a maxillary denture
base
● Denture-associated pathology
Key Facts
● Second most common oral ulcer (behind traumatic ulcers)
● Exclusive to unkeratinized tissue
● Vary in size (usually <1 cm), either solitary or widely
distributed
● Associated with Bechet’s disease and Crohn’s disease
● Favors younger patients
● Unilocular or multilocular radiolucencies in tooth-bearing
areas
● Hyperparathyroidism must be ruled out
● Fissuring mass of the gingiva or alveolar mucosa
● Pebby surface texture
● May be associated with Candidiasis
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Condition Clinical Image Description Key Facts

Mucocele ● Caused by traumatic injury to minor ● Mucous extravasation phenomenon of the lower labial
salivary glands (lower lip) mucosa
● Blue, dome-like, fluctuant swelling

Reactive Processes

Nicotinic stomatitis (Smoker’s ● Benign, reactive hyperkeratosis caused ● Exclusive to the hard palate (not soft palate, which would
palate) by the heat of cigarettes suggest epithelial dysplasia)
● Represents damage to minor salivary ● Presents as gray or white papules with red dots in the center
glands

Peripheral ossifying fibroma ● Fibrous hyperplasia with metaplastic ● May originate as a traumatic fibroma
osseous tissue
● Large mass on a palatal alveolar process

Pyogenic granuloma ● Exuberant, reactive hyperplasia of ● Gingiva is most common, but not exclusive (site of chronic
granulation tissue irritant, inflammation)
● Painless, raised, red, raspberry-like ● Can arise during periods of hormonal flux (pregnancy)
mass ● Ulcerated, may bleed
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Condition Clinical Image Description Key Facts

Ranula ● Caused by traumatic injury to minor ● Mucous extravasation phenomenon (mucocele) of the floor
salivary glands (floor of the mouth) of the mouth
● Blue, dome-like, fluctuant swelling

Reactive Processes

Traumatic bone cyst ● Intraosseous pseudocyst associated with ● Favors younger patients
trauma ● Associated teeth are vital
● Well-defined radiolucency with scallops
around the apices of teeth
● Painless, incidental finding

Traumatic fibroma ● Exuberant, benign fibrous hyperplasia ● Among most common benign reactive hyperplastic lesions
associated with trauma ● May develop to a peripheral ossifying fibroma
● Raised, soft/firm mucosal nodule ● Analogous to a dermal keloid
comprised of fibrous tissue

Traumatic ulcer ● Ulceration caused by direct physical injury ● Most common oral ulceration
to oral mucosa, associated with trauma ● Usually isolated, solitary
● Initially painful ● Do not predispose to oral cancer