test 5 quick study guide.pdf

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Oral Pathology 2006

Test 5Quick Study Guide 1 of 22

Bone Pathology

Osteogenesis Imperfecta Heterogeneous group of heritable connective tissue disorders Secondary to mutations in either the COL1A1 or COL1A2 genes, which guide type 1 collagen formation Dominant and recessive inheritance patterns Clinical Features-- Four major types:

o Type I Most common and least severe form, moderately severe bone fragility, blue sclera,hearing loss before age 30, some show opalescent teeth

o Type II Most severe form, extreme bone fragility with frequent fractures, blue sclera, someopalescent teeth

o Type III Most severe form beyond perinatal period, moderately severe bone fragility, bluesclera, kyphoscoliosis, some opalescent teeth

o Type IV Mild to moderate bone fragility, blue sclera fades with age, bone fracturesdecrease after puberty, some opalescent teeth

Histology-- Cortical bone appears attenuatedo Reduced bone matrix productiono Bone architecture remains immature throughout life, with woven bone failing to transform to

lamellar bone

Treatment--Management of fractureso Dental management of opalescent teetho Prognosis variable, depending upon the type

Osteopetrosis (Albers-Schonberg Disease, Marble Bone Disease) Hereditary disorder characterized by increased bone density Secondary to defect in osteoclastic function Two distinct forms :

o Infantile (malignant) form: Normocytic anemia Symptoms secondary to cranial nerve pressure Pathologic fracture Skull deformities, hypertelorism Delayed tooth eruption Autosomal recessive

o Adult (benign) from; Milder, typically detected upon routine radiograph No anemia No cranial nerve compression Uncommon pathologic fracture Osteomyelitis Autosomal dominant

Histology-- Numerous osteoclastso Lack of Howships lacunaeo Dense bone formation

treatment and Prognosis-- Supportiveo Transfusions, antibioticso Infantile form results in death during the first decadeo Better prognosis for adult form

Cleidocranial Dysplasia Hereditary bone defect of the bone and clavicles Secondary to defect in the CBFA 1 gene, which guides osteoblastic differentiation Frontal and parietal bossing Ocular hypertelorism Mid-face hypoplasia Open fontanels and wormian bones Hypoplasia or absence of clavicles Oral- Numerous unerupted and supernumerary teeth Histology-- Lack of secondary cementum (failure of the teeth to erupt?)


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Treatment-- None for skeletal anomalieso Extractions, surgical exposure of unerupted teeth with orthodontic treatmento Orthognathic surgery

Focal Osteoporotic Marrow Defect Harmless process that may mimic pathology May be secondary to aberrant bone regeneration following extraction, persistence of fetal marrow,

and/or marrow hyperplasia

F>M Posterior mandible (70%) Asymptomatic Ill-defined radiolucency with fine trabeculations Histology-- Normal bone; Cellular hematopoietic marrow Treatment-- Incisional biopsy may be necessary to establish Incisional diagnosis

o No other treatment is necessary; Excellent prognosisIdiopathic Osteosclerosis

Focal area of bone density of unknown etiology Often confused with condensing osteitis Age 20 Age 20-40 40 Mandibular first molar area Symptomatic; Well defined area of radiopacity

Histology-- Dense lamellar bone; Little or no inflammation Treatment-- None necessary

o Lesion may regress with timePagets disease of Bone (Osteitis Deformans)

Disease of bone characterized by abnormal resorption and deposition of bone Unknown etiology; Recent evidence suggests a possible viral etiology M>F; Older individuals Monostotic vs. polyostotic Thickened, enlarged, and weakened bones Often painful (Bone pain is a common complaint) Maxilla > Mandible (leontiasis ossea)

Leonitis Ossea= midface deformity resulting in lion-facies due to inv. of maxilla Enlarged alveolar ridges May see spacing of the teeth Radiograph

o Early Decreased radiodensity and altered trabeculationo Later Patchy, sclerotic bone (cotton wool)o Hypercementosis

Lab Findingso Increased serum alkaline phosphataseo Normal calcium and phosphorous levelso Elevated urinary hydroxyproline

Histology-- Osteoblastic formation and osteoclastic resorption of boneo Basophilic reversal lines (mosaic or jigsaw pattern)

Treatment and Prognosis--Analgesics for bone paino Parathyroid hormone antagonistso Slowly progressiveo Osteosarcoma develops in about 1% of patients

My Notes: Need to be able to differentiate b/t bone pain and patchy radiolucencyLangerhans Cell Disease (Langerhans Cell Histiocytosis, Histiocytosis X) Neoplastic proliferation of Langerhans cells (actually a blood disorder) Langerhans cells Dendritic mononuclear cells normally found in the epidermis, mucosa, lymph nodes, and

bone marrow

Three forms: (historical purposes @ best)o Letterer-Siwe disease Acute, disseminatedo Hand-Schuller-Christian disease Chronic, disseminatedo Eosinophilic granuloma

M>F; Less than 10 years old


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Solitary or multiple bone lesions Floating in air radiographMay mimic periodontal disease Histology--Diffuse infiltrate of pale mononuclear cells (CD1a+, S100)

o Eosinophilso Birbeck granules seen on electron microscopy

Treatment-- Curettage is often adequateo Radiationo Chemotherapy for disseminated formo Prognosis of bone lesions alone is goodo Poorer prognosis for acute disseminated form

My Notes= Know CD1a+ & S100 positivity; also remember it can involve skull and hipCentral Giant Cell Granuloma

Non-neoplastic lesion of the jaws Significance lies in differentiation from brown tumor of hyperparathyroidism and association with

cherubism F>M Aggressive vs. non Aggressive (Ignore says Dr A.) Typically painless, expansile Mandible, anterior to the first molar (In Cherubism, I is posterior, too!) May cross the midline

Unilocular to multilocular radiolucency Histology-- Spindle cell proliferation with variable numbers of multinucleated giant cells

o Erythrocytic extravasation and hemosiderin depositiono May see osteoid

Treatment= Curettageo Recurrent lesions sometimes require more radical surgeryo Good prognosis, although a 15-20% recurrence is reported

My Notes: More than one CGCG bet on hyperthyroidismCherubism (KNOW SLIDE) Rare autosomal dominant condition of the jaws Name is derived from the resemblance of the patients to angels depicted in Renaissance paintings Children Painless bilateral expansion Usually involves the posterior mandible Eyes turned up to heaven appearance Multilocular, expansile radiolucencies Noonan-like syndrome Histology-- Giant cell granuloma, with more loosely arranged stroma

o Giant cells are focally aggregatedo Eosinophilic hyaline angiopathy

Treatment= Often shows remission and involution after pubertyo Treatment is debatable and dependant upon the individual patients needs

My Notes= tx. is controversial b/c it regresses on its own and TREATMENT MAKES IT WORSE;Not necessary to biopsy; may try intralesional corticosteroids but not necessary

Traumatic Bone Cyst (Simple Bone Cyst) Benign empty or fluid-filled bone cavity of uncertain pathogenesis Actually not a true cyst (no epithelial lining) Trauma-hemorrhage theory is probably the most popular of potential explanations; Others include

ischemic necrosis, growth disturbances Trauma-Hemorrhage theory= trauma to the bone, insufficient to cause fracture leads tointraosseous hematoma with lack of organization & repair resulting in liquefaction leaving a cystic

defect

M>F; Second decade Most involve the mandible Typically asymptomatic Radiographic appearance= Radiolucency with sharply or ill-defined margins

o Scalloping between roots Histology= Thin lining of fibrovascular connective tissue along the bony wall ; No epithelial lining


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Treatment- Surgical exploration for establishment diagnosis; Rapid resolution is expected after thisprocedure

My Notes: Upon biopsy, you see nothing but an empty hole with no lining; in biopsy report to lab,clinical info is just as impt. as the lesion itself

Benign Fibro-Osseous Lesions of the Jaws Nonspecific term used to describe a number processes that are characterized by fibrous connective

tissue and mineralized tissue

Due to similarity in histopathologic features, radiographic and/or clinical correlation is necessary toestablish a definitive diagnosis

Three main processes:o Fibrous dysplasiao Cemento-osseous dysplasia My Notes: Know all of these but especially Periapical cemento-osseous dysplasiao Ossifying (cemento-ossifying) fibroma

Fibrous Dysplasiao Developmental process secondary to a postzygotic mutation in the GNAS 1 geneo Three forms:Monostotic or polyostotic

Polyostotic Jaffe-Lichtenstein syndrome Polyostotic McCune-Albright syndrome

o Clinical Features= Typically affects children during the first or second decade

M=Fo Monostotic

Slowly growing enlargement of a single bone Frequently involves the jaws Ground glass radiograph

o Jaffe-Lichtenstein syndrome Typically involves the long bones Pathologic fracture, pain, deformity

o McCune-Albright syndrome Polyostotic features Caf-au-lait pigmentation Multiple endocrinopathies

o Histology= Cellular fibrous stroma Irregular shaped trabeculae of woven bone (Chinese characters) Skull and jaw lesions mature differently

o Treatment= Often stabilizes with puberty Surgical resection or recontouring of bone lesions; Generally a cosmetic issue

Cemento-Osseous Dysplasiaso Fibro-osseous lesions that occur in the tooth-bearing areas of the jawso Three forms depending upon clinical presentation:

Periapical cemental (cemento-osseous) dysplasia Focal cemento-osseous dysplasia Florid cemento-osseous dysplasia

My Notes: Know all of these but especially Periapical cemento-osseous dysplasiao Periapical cemental dysplasia

African-American females (14:1 female predilection) Mandibular anterior area, asymptomatic Radiolucent to mixed appearance Assoc. teeth are vital Early lesions resemble radicular cyst or dental granuloma Eventually becomes a circumscribed, dense calcification surrounded by a

radiolucent rim

o Focal cemento-osseous dysplasia F>M; Whites>Blacks Posterior mandible Solitary; Asymptomatic

o Florid cemento-osseous dysplasia Adult black females


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Often symmetric involvement of all four quadrants My Notes= if you biopsy, comes out like coffee grounds; As these masses get older, theymineralize & get very susceptible to inflamm. & may eventually get osteoslerosis; Problems with

osteomyelitis; Oral Hygiene is very impt.o Histology= Early stage-- Fibroblastic proliferation; Possibly small foci of osteoid formation

Later stage= Greater deposition of bone or cementum-like materialo Treatment= None for the milder presentations; Florid presentations become problematic with

ridge resorption Sequestration and osteomyelitisOssifying Fibroma (Cemento-Ossifying Fibroma)

Neoplasm of fibro-osseous differentiation Amount of cementum-like and osseous-like is variable from lesion to lesion and can be used determine the

terminology (ossifying, cementifying, or cemento-ossifying)

F>M; Adults Mandibular molar/premolar area Typically an asymptomatic swelling Radiographic appearance= Well defined, Unilocular; Varying degrees of opacity; Bowing of inferior

cortex; Uncommon to see root resorption or divergence

Histology= Most are unencapsulated; Fibrous tissueo Calcified component of osteoid/bone and/or cementum-like material

Treatment= Enucleation (lesion will shell out in one potato-like mass)o

Large lesions may require more extensive surgeryo Rare recurrence, no malignant potential

Osteoma Benign tumor of compact or cancellous bone Predominantly restricted to the bones of the craniofacial skeleton May occur on the surface (periosteal) or within medullary bone (endosteal) Young adults Asymptomatic Sinus lesions Sinusitis, headache, exopthalmos Radiograph Circumscribed, sclerotic mass Histology=

o Compact Dense lamellar bone with minimal marrowo Cancellous Trabeculae of bone with fibro-fatty marrow

Treatment= Small lesions Observationo Larger lesions Surgical excisiono Recurrence is unexpected; No malignant potential

My Notes= Multiple osteomas is a char. of Gardners Syn.; Periosteal form common at angle ofmd.

Gardner Syndrome Autosomal dominant condition characterized by colorectal polyps (adenomatous), osteomas, epidermoid

cysts of the skin, and desmoid tumors

Chief significance of Gardner syndrome lies in the potential for the potential for adenocarcinoma ofthe colon

Osteomas, particularly of the jaws and bones of the skull Epidermoid cysts, Multiple odontomas and impacted, supernumerary teeth Histology= Compact type of osteomas

o Epidermoid cystso Adenomatous polyps of the colon

Treatment= Prophylactic colectomyo Cosmetic removal of osteomas and epidermoid cystso Prognosis Without appropriate treatment, the incidence of colorectal carcinoma approaches

100% in the elderly

Osteoblastoma Benign lesion of bone arising from osteoblasts Significant radiographic and histopathologic similarities with cementoblastoma (next topic) M>F; Under age 30 2 - 4 cm Continuous pain, unrelieved by salicylates (this is a dist. char. b/t this and fibro-osseous lesions)


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Radiolucency with patchy mineralization Histology= Basophilic osteoid (blue bone) with prominent osteoblastic rimming

o Fibrovascular stroma; Osteoclasts Treatment= Local excision or curettage; Favorable prognosis, with few recurrences Note: Book discusses osteoid osteoma but Dr A. said dont worry about it

Cementoblastoma (True Cementoma)

Odontogenic neoplasm; Probably the only true neoplasm of cementum Many similarities to osteoblastoma, warranting discussion with bone pathology Mandibular (1st) molar region Variable pain Expansile Calcified mass with radiolucent rim associated with the root of a tooth Histology= Similar to osteoblastoma, but fused to the tooth root

o Sheets or trabeculae of mineralized materialo Uncalcified matrix arranged in radiating columns at the periphery

Treatment=Tooth extraction (surgical) along with the attached masso Removal of mass and involved root, along with endodontic therapyo Excellent prognosis with removal of the entire lesion

Osteosarcoma (Osteogenic Sarcoma)) Malignancy of mesenchymal cells that are characterized by the ability to produce osteoid or bone

Most common malignant tumor of bone M>F; Avg. age 33 for jaw lesions Most occur in the area of the knee Swelling, pain, parasthesia, loosening of teeth ; Painful Variable radiographic appearance:

o Spiking root resorptiono Sunburst or sunray appearanceo Early widening of PDL early sign= symmetrical widening of pdl

Histology= Direct production of osteoid by mesenchymal cells (essential criterion)o Variants = Osteoblastic, Chondroblastic, Fibroblastic, Small cello Must have osteoid formation for diagnosis

Treatment= Radical resection, often with chemotherapy, radiation, or botho Up to 80% survival with radical surgeryo Death most often secondary to uncontrolled disease

Peripheral (Juxtacortical) Osteosarcoma Variant of osteosarcoma that arises from the surface of bone Much better prognosis than intramedullary osteosarcoma Parosteal and periosteal typesMy Notes: Periostealsessile and you get elevation of the periosteum, easier to tx; parostealattached to cortex w/o elevation of periosteum

Chondrosarcoma Malignant tumor of cartilage formation Rarely affects the jaws M=F; Avg. age ? Painless mass or swelling Separation or loosening of teeth Poorly defined radiolucency Scattered radiopaque flecks May see the sunburst pattern (But more common in osteosarcoma) Mesenchymal Chondrosarcomavariant of chondrosarcoma in younger pts.just know that it is a high-

grade malignancy that basically is a chondrosarcoma along w/ another tumor

Histology= Lobular pattern of cartilage formation with varying degrees of maturation and cellularityo Three histologic grades, 1-3o My notes: can see calcification but the tumor cells arent making osteoid

Treatment= Radical resectiono Poorer prognosis for jaw lesions than osteosarcoma?o Low potential for metastasis, death usually occurs by direct extension


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My Notes: Chondroma= benign tumor of cartilage that isnt common in the oral-maxillofacialregion (maybe TMJ but not jaws)if the lab report says this it is a mis-diagnosis and should be

treated as a low-grade chondrosarcoma

Ewings Sarcoma Controversial lesion of bone, probably neuroectodermal in origin Distinct genetic translocation Distinct-- t(11;22) ***Know*** Uncommon tumor of the jaws M>F; Children adolescents Swelling with intermittent pain Parasthesia and loosening of teeth Lytic bone destruction with ill-defined margins Onion-skin periosteal reaction Histology=Sheets or lobules of small round cells

o Blue cell tumoro Intracytoplasmic glycogeno CD99 positivity

Treatment=Surgery, radiotherapy, and multi-agent chemotherapyo 40-80% survival, possibly with recent 80 improvemento Metastasis to lungs, liver, lymph nodes, and other bones My Notes: fever, leukocytosis and increased ESR

Metastatic Tumors to the Jaws Most common malignancy involving bone Hematogenous spread (Bastons plexus) Uncommon in the jaws? Breast, lung, thyroid, prostate, and kidney Older patients Mandible>Maxilla Pain, swelling, loosening of teeth, parasthesia Old TR questionsbypasses lungs via Bastons plexus; more common in mand. May mimic periodontal disease Moth-eaten radiographic appearance Breast and prostate may show mixed radiolucent/opaque pattern Histology= Variable, depending upon the primary tumor

o Must have histologically identical primary tumor elsewhere Treatment= Solitary lesions may be treated by excision or radiation

o Treatment is often palliativeo Poor prognosis, with patients usually dying within 1 yearMy Notes: Differential Diagnosis for Blue-Cell Tumor= Ewings Sarcoma, Lymphoma,Neurofibroma, Rhabdomyosarcoma, etc.

Odontogenic Cysts

Dentigerous Cyst The most common developmentalodontogenic cyst Originates secondary to the separation of the follicle from the crown of an unerupted tooth (** By

Hydrostatic Pressure)

M>F; 2nd & 3rd decades Mandibular 3rd molars most common, followed by maxillary canines Typically asymptomatic or a painless expansion Radiograph Unilocular radiolucency (Unilocular RL 9/10 times, its a Dentigerous Cyst) Affected tooth may be displaced May see root resorption of adjacent teeth Histology-- Thin lining of cuboidal to non-keratinizing squamous epithelium

o Uninflamed fibrous connective tissue wallo Some cysts may become secondarily inflamed

Treatment= Enucleation and removal of the involved tootho Marsupialization of larger cysts

Rare recurrence; Rare cysts transform to squamous cell carcinoma or mucoepidermoid carcinoma


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Eruption Cyst (Eruption Hematoma)

Soft tissue counterpart to the dentigerous cyst Occurs within the soft tissue overlying the alveolar bone Under 10 years old Typically involves mandibular molars NO RADIOLUCENCY Soft, translucent swelling of the gingival mucosa overlying an unerupted tooth Histology=Thin layer of non-keratinizing squamous epithelium lining the inner portion of the soft tissue Treatment=Typically no treatment is necessary

o Excision of the soft tissue may permit speedy eruption of the involved toothPrimordial Cyst

Traditionally, the primordial cyst is defined as a cyst that develops in place of a missing tooth Most are histologically diagnosed as odontogenic keratocyst

Odontogenic Keratocyst

Distinct odontogenic cyst with specific histologic features and clinical behavior Arises from cell rests of the dental lamina Also associated with nevoid basal cell carcinoma syndrome M>F; 10-40 years Mandible > Maxilla Rarely cause expansion

Unilocular to multilocular radiolucency (First in differential for multilocular radiolucency of posteriormandible. Unilocular is most likely to be Dentigerous cyst but may be an odontogenic keratocyst.

Also, OKC may be seen in anterior maxilla of older pts.)

Histology= Uniform layer of stratified squamous epitheliumo Corrugated layer of parakeratino Hyperchromatic palisaded basal layer (**KNOW ALL)

Treatment= Enucleation and curettageo Recurrence rate of about 30%, so careful follow-up is mandatory (Due to propensity for

multiple daughter cysts)

o May need to rule out nevoid basal cell carcinoma syndromeOrthokeratinized Odontogenic Cyst

Not necessarily a clinical type of cyst, but an odontogenic cyst that microscopically has anorthokeratinized lining

Markedly different clinical behavior from the odontogenic keratocyst Clinical Features= Nonspecific

o M>F; Young adultso Most involve the mandibular third molars

Histology=Stratified squamous epithelium that exhibits a prominent layer of orthokeratin My Notes: No palisaded basal layer; completely different from OKC Treatment= Enucleation and curettage

o Few recurrences and not associated with nevoid basal cell carcinoma syndromeNevoid Basal Cell Carcinoma Syndrome (Gorlin Syndrome)

Autosomal dominant syndrome secondary to mutations in the PTCH gene High penetrance and variable expressivity Features multiple basal cell carcinomas of skin, odontogenic keratocysts, rib and vertebral

anomalies, and intracranial calcifications

Clinical=Frontal and temporoparietal bossingo Ocular hypertelorismo Multiple and early basal cell carcinomaso Palmar/plantar pittingo Bifid ribs and other skeletal anomalieso Calcified falx cerebrio Multiple odontogenic keratocysts

Histology=Similar to other odontogenic keratocysts, with increased numbers of daughter cystso Typical basal cell carcinomas

Treatment and Prognosis=Multiple surgeries for removal of cysts and basal cell carcinomas Recurrence and prognosis similar to that of odontogenic keratocysts and basal cell carcinomas


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My Notes: PCTH is a tumor suppressor gene; Remember that its Autosomal Dominant; Know the chieffeatures, especiallyBIFID RIB, SCOLIOSIS, MULTIPLE BASAL CELL CARCINOMAS (SKIN),

MULTIPLE JAW CYSTS (OKC), & INTRACRANIAL CALCIFICATIONS; Prognosis depends on behavior of

skin tumors, usually not life threatening

Gingival Cyst of the Newborn (Dental Lamina Cyst of the Newborn) Superficial, keratin-filled cysts on the alveolar mucosa Arise from remnants of the dental lamina Maxilla > Mandible Multiple small papules on the alveolar processes of neonates Histology=Thin keratinizing stratified squamous lining

o Keratinaceous debris within the lumen Treatment= No treatment necessary; Cysts spontaneously involute My Notes: appear like Bihns Nodules & Epstein Pearls; Seldom noticed/ biopsied; keratin-filled

Lateral Periodontal Cyst

Developmental (not inflammatory) cyst Probably arises from rests of the dental lamina M>F; Over age 30 M>F; Over age 30 Mandibular canine/premolar area Well circumscribed radiolucency lateral to the root of a vital tooth multilocular=botryoid

Histology= Thin lining of squamous epithelium with thickenings of clear cells (occasionally)o Un-inflamed fibrous connective tissue wall

Treatment and Prognosis:Conservative surgical enucleationo Occasional recurrence of the botryoid variant

MyNotes: Remember its not inflammatory (Lateral Radicular is though); Almost always b/t md

premolar and canine; Radiographic features not diagnostic (could be OKC or an inflammatory cyst);

Clear cells are glycogen rich; Recurrence of Botryoid variant probably due to incomplete enucleation.

Gingival Cyst of the Adult Soft tissue counterpart of the lateral periodontal cyst Derived from rests of the dental lamina 5th 6th decade Mandibular canine-premolar area Blue to blue-gray dome-shaped swelling Histology=Thin lining of non-keratinizing stratified squamous epithelium

o Focal plaques and nests with clear cells Treatment and Prognosis=Simple excision; Excellent prognosis

My Note: No way is this a mucocele, b/c its on attached gingiva; May see cupping of alveolarbone; May or may not see clear cells

Calcifying Odontogenic Cyst (Gorlin Cyst) Uncommon cyst, sometimes associated with other odontogenic tumors If no cystic component is present, odontogenic ghost cell tumor or dentinogenic ghost cell tumor is

more appropriate

Avg. age 33 Maxilla = Mandible, often involving the maxillary incisor-canine region May be peripheral (outside bone) Unilocular radiolucency, often with radiopaque structures within the lesion May see root resorption or divergence Histology= Lining of odontogenic epithelium with cuboidal or columnar basal cells

o Overlying epithelium more loosely arrangedo Uninflamed fibrous capsuleo Ghost cellso Dysplastic dentin (dentinoid) may be seeno Cystic variants

Intraluminal proliferation Associated with odontomas

o Neoplastic (solid) variant---May Resemble Ameloblastoma but distinguished by presence ofGhost cells and juxtglomerular dentinoid

o Aggressive/malignant variant


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Treatment = Simple enucleationo Best prognosis for the peripheral and cystic typeso Prognosis will depend upon other tumors that COC may be associated with

Glandular Odontogenic Cyst (GOC) Rare odontogenic cyst that shows features of glandular differentiation Also referred to as sialo-odontogenic cyst The pathogenesis of this lesion is unknown Adults, mandible Larger lesions capable of bony expansion Well- defined multilocular radiolucency with a sclerotic rim Histology=Squamous epithelial lining

o Hobnail appearance to the luminal epithelial cellso Mucous cells and duct-like structures may be seen

Treatment= Very few reported cases, so definitive information regarding prognosis is lackingo Thorough curettage is the current treatment of choiceo 30% recurrence has been reported

My Notes: considered odontogenic but is really glandular; Common in ant mandible and CANCROSS MIDLINE!; may or may not get pain

Carcinoma Arising in Odontogenic Cysts Carcinoma arising from odontogenic epithelium

May arise de novo or from malignant transformation of a pre-existing cyst or neoplasm Must rule out the possibility of metastatic carcinoma M>F, Older patients Pain, swelling, parasthesia Irregular, ragged radiolucency Histology=Typically a well differentiated squamous cell carcinoma

o May see transition from the original cyst or neoplasm Treatment and Prognosis= Excision with or without radiation or chemotherapy

o Local metastasis is infrequento Probably a 50% 5-year survival rate

My Notes: Most likely to undergo malignant transformation is residual PA cyst (others include

Dentigerous cyst, OKC, & lateral periodontal cyst); Chemo may buy them ~1 month

Odontogenic Tumors

3 main categories:o Odontogenic epithelium without odontogenic ectomesenchymeo Odontogenic epithelium and odontogenic ectomesenchyme with or without dental hard tissueo Predominantly odontogenic ectomesenchyme without or with a minor element of odontogenic

epithelium

My Notes: these usually involve the inner dental epithelium; one tumor arises from the stratumintermedium; Reversed polarity= nucleus at one end of cell; Palisaded= cells lined up like tombstones

Tumors of Odontogenic Epithelium

Ameloblastoma

The most common of the odontogenic epithelial neoplasms Arises from odontogenic epithelium 3 clinicoradiologic situations

o Conventionalo Unicystico Peripheral

Conventional AmeloblastomaClinical Featureso M=F, Avg. age 34o Asymptomatic swelling and expansiono Multilocular radiolucency, often associated with an unerupted tooth

Desmoplastic varianto Predilection for anterior regions of jawso Radiographic resemblance to fibro-osseous lesionso


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Histology=o Follicular pattern

Islands of epithelium resembling the enamel organ with peripheral palisading andreverse polarity

Central area resembling stellate reticulumo Plexiform pattern=

Anastomosing cords or sheets of cells Peripheral palisading and reverse polarity

o Acanthomatous pattern Squamous metaplasia within the tumor islands Keratin formation with occasional keratin pearls

o Granular cell pattern Cells within the islands are characterized by a granular cytoplasm

o Desmoplastic pattern Small islands and cords of odontogenic epithelium Dense fibrous stroma

o Basaloid pattern Nests of basaloid cells Peripheral islands more cuboidal than columnar

o Treatment and Prognosis

Enucleation to en bloc resection 15% recurrence with marginal resection, 50-90% with curettage My Notes: Painless and benign but very aggressive; usually much bigger than you see on x-ray;SOAP BUBBLE and HONEYCOMB x-ray; May resemble Botryoid Lateral periodontal cyst but will

usually be bigger (this is why you biopsy); Desmoplastic type- fibrous outlier that usually shows up

in anterior maxilla; AMELOBASTOMA CAN ALSO CROSS MIDLINE; Just Know the different types

exist; ONLY HISTO TO KNOW= Palisading Nuclei, Reverse Polarity, Core looks like stellate

reticulum.

Unicystic Ameloblastoma Controversy regarding its definition Some feel that the lesion must be unilocular Others believe that multilocular is acceptable if the internal aspect is cystic 10-15% of intraosseous ameloblastomas Less aggressive lesion with significantly lower recurrence rate Younger patients Mandible, usually posterior Asymptomatic or painless swelling Circumscribed radiolucency Histology=Cystic lining shows features of ameloblastic epithelium

o Variants= Luminal, Intraluminal, Mural Treatment= Curettage

o More aggressive treatment may be indicated for mural subtypeo Overall better prognosis than conventional ameloblastoma

My Notes: Usually a circumscribed radiolucency that surrounds the crown of an unerupted

mandibular 3rd molar or sharply defined radiolucent area thought to be a primordial, radicular, orresidual cyst; HISTO= PALISADING, REVERSE POLARITY & STELLATE RETICULUM; Higher

recurrence probably due to curettage with mural type.

Peripheral Ameloblastoma (Extraosseous) Uncommon variant Probably arises from submucosal rests of odontogenic epithelium or from the basal odontogenic layer of

the surface epithelium

Middle aged patients; Posterior mandible Painless gingival swelling (resembles a fibroma) May superficially erode bone, but overall no radiographic evidence of intraosseous lesion Histology= Histologically resembles intraosseous ameloblastoma, with all variants possible

o 50% will connect with the surface epithelium Treatment=Local excision ; Much less aggressive than the intraosseous counterpart

o If the lesion recurs, re-excision is curative


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Malignant Ameloblastoma and Ameloblastic Carcinoma

Ameloblastic carcinoma Cytologically malignant ameloblastoma Malignant ameloblastoma Cytologically benign lesion showing metastasis Avg. age 30 Metastatic site primarily the lungs, often after 10 years Ameloblastic carcinoma will show ill defined, destructive lucency Histology=

o Malignant ameloblastoma Identical to conventional ameloblastomao Ameloblastic carcinoma Primary and metastatic tumor with histologic features of malignancy

Treatmento Prognosis is poor for both malignant ameloblastoma and ameloblastic carcinoma

--My Notes: Remember that the primary metastatic site is the lungs; Ameloblastic Carcinoma x-

ray has ill-defined margins & cortical destructionCalcifying Epithelial Odontogenic Tumor (Pindborg Tumor)

Uncommon tumor of uncertain histogenesis Probably arises from stratum intermedium of enamel organ Posterior mandible; adults Often associated with impacted tooth Radiolucent, with or without driven-snow radiopacities; May show cupping resorption Histology=Islands, strands, or sheets of polyhedral epithelial cells

oAmyloid (congo red positive)

o Liesegang ringsAmyloid islands calcifying to form concentric rings Treatment=Excision with a narrow rim of surrounding bone

o Good prognosis, with a 15% recurrence rateSquamous Odontogenic Tumor

Benign odontogenic tumor that arises either from rests of the dental lamina or the rests of Malassez The tumor tends to originate from within the periodontal ligament M=F, Avg. age 37 Painless or slightly painful swelling Mobility of associated teeth Triangular radiolucency Histology= Islands of bland-appearing squamous epithelium

o Microcystic vacuolization and individual cell keratinizationo SOT-like proliferation

Treatment= Conservative excision or curettageo Prognosis - goodo A few recurrences have been reported, but these have been controlled with further these local

excision

--My Notes: SOT-Like proliferations seen in wall of dentigerous cyst; Biggest significance is indifferentiating b/t this and SCC; NO PERIPHERAL PALISADING ON HISTO

Tumors of Odontogenic Epithelium with Odontogenic Ectomesenchyme (probably from dental papilla)

Ameloblastic Fibroma

Mixed odontogenic tumor with both neoplastic epithelial and mesenchymal tissues Many of these may actually represent early developing odontomas M>F, First 2 decades Posterior mandible; Many associated with an unerupted tooth Unilocular or multilocular radiolucency Histology= Mesenchymal tissue resembling the dental papilla (pulp)

o Odontogenic epithelium in islands or cords (like Ameloblastoma)o Epithelial portion resembles developing enamel organ

Treatment=Initial conservative excisiono Aggressive curettage for recurrent lesionso Prognosis: good, although a 20% recurrence rate has been reported in some series

Adenomatoid Odontogenic Tumor (AOT) Originally included in the variants of ameloblastoma Derived from the enamel organ epithelium


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May produce dentinoid material, rarely enamel matrix F>M; Most occur during the second decade Anterior maxilla Large lesions cause a painless expansion Circumscribed radiolucency involving the crown of an unerupted tooth Extends beyond the CEJ May contain snowflake calcifications Histology= Thick fibrous capsule

o Aggregates of spindle-shaped cells frequently in a whorled arrangemento Variable numbers of duct-like structureso May contain small foci of calcification

Treatment= Thick capsule allows for simple enucleationo Probably doesnt recur

My Notes: if you see a slide on the test that looks like this, answer will be dentigerous cyst b/cit is more likely;; Extension beyond CEJ is a clue it is AOT; Usually around impacted canine

Odontoma Most common odontogenic tumor Two types, based upon the arrangement of the Two components of odontogenesis

o Compoundo Complex

First two decades

Complex Posterior maxilla or mandible Compound Anterior maxilla Large lesions may cause painless expansion Radiograph=

o Complex Calcified mass with the density of tooth structureo Compound Multiple tooth-like structures with radiolucent rim

Histology= Complex Haphazard arrangement of hard dental tissueso Compound Multiple, small single-rooted teetho May see ghost cells

Treatment= Local excision; Excellent prognosis My Notes= there is a difference b/t the radiodensity of bone and tooth (osteoma vs. odontoma) ONTEST ODONTOMA WILL BE THE BEST ANSWER!!!; Ghost cells more common w/ calcifying odontogenic

cyst

Tumors of Odontogenic Ectomesenchyme with or without included Odontogenic Epithelium

Neoplastic portion is entirely ectomesenchymeOdontogenic Fibroma

Uncommon lesion with two described histologic subtypes, although this has recently been questionedo Simple typeo WHO type

F>M, Avg. age 40 Maxilla, anterior to the first molar Expansion, loosening of teeth Unilocular to multilocular radiolucency; Root resorption or divergence Histology=

o Simple type= Background of fine collagen fibrils with stellate fibroblasts May see small foci of odontogenic epithelium

o WHO type= More cellular fibrous connective tissue Strands and nests of odontogenic epithelium

Treatment= Enucleation and vigorous curettageo Limited growth potential ; Good prognosis

My notes: there is also a peripheral type that shows up as a bump on the gums (3Ps morelikely); You will see a radiolucency and also a dimple on the palate

Peripheral Odontogenic Fibroma Soft tissue counterpart to the odontogenic fibroma Most similar to the WHO type Slow growing gingival mass


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May cause tooth displacement Radiograph may show areas of calcification within the soft tissue mass Histology= Cellular fibrous connective tissue

o Strands and nests of odontogenic epitheliumo Variable calcification

Treatment= Local excisiono Good prognosis with few recurrences

Myxoma (Odontogenic Myxoma) Considered to be of odontogenic origin, resembling the odontogenic ectomesenchyme Histologically resembles the mesenchymal portion of a developing tooth M=F, Age 25-30 Mandible > Maxilla Asymptomatic expansion Soap bubble radiolucency Histology= Gelatinous, loose (myxoid) background

o Variable islands of odontogenic epitheliumo Fibromyxoma, myxofibroma

Treatment= Larger lesions require more extensive resectiono Infiltrative tumor, with a recurrence potential of 25%o Despite recurrence, the prognosis is good Despite recurrence, the prognosis is good

My Notes: DIFFERENTIAL DIAGNOSIS FOR POSTERIOR MANDIBLE MULTILOCULARRADIOLUCENCY= 1) AMELOBLASTOMA, 2) OKC, 3) MYXOMA, If expansile, probably Myxoma or

Ameloblastoma;Trabeculae line up @ right angle to the periphery on CT or occlusal radiograph;

Fibromyxoma & Odontogenic Myxofibroma are variants of Myxoma w/ more collagen so not as

gelatinous (kind of a hedge b/t central osseous fibroma and myxoma)not too impt.

Dermatologic Diseases

Ectodermal Dysplasia Hereditary condition of defective ectodermal development Over 150 subtypes, with the best known being hypohidrotic ectodermal dysplasia (X-linked) Autosomal dominant and recessive forms are also present M>F Heat intolerance (due to no sweat glands) Sparse hair, dystrophic nails Wrinkled, hyperpigmented periocular skin Midface hypoplasia, protruberant lips Oral= Xerostomia

o Hypodontia with malformed crown shapes (oligodontia and anodontia also possible) Histology= Skin biopsies show decreased numbers of sweat glands and hair follicles

o those adnexal structures that are present are hypoplastic Treatment= Genetic counseling; Prosthetic replacement of dentition My Notes: can involve any ectodermally derived structures; Xerostomia b/c salivary glands areectodermally derived; HED most common type; HED most likely cause of total anodontia; Usually in

males (X-Linked) but if in females, less severe due to lionization phenomenon; No problem with bone somay consider implants but must consider age;

White Sponge Nevus (Cannons Disease) Autosomal dominant genodermatosis caused by mutations in keratin genes 4 and 13 The defect results in abnormal keratinization of the mucosa Bilateral, thickened, white, corrugated plaques primarily affecting the buccal mucosa Most mucosal sites are also susceptible Histology= Prominent hyperparakeratosis and acanthosis

o Cytoplasmic clearingo Eosinophilic perinuclear condensation (readily seen on cytologic smear)

Treatment= None necessaryo Prognosis good


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My Notes: Genodermatosis= genetically determined skin disorder; Keratin 4 & 13 are expressedb/t spinous and basal layer, these are defective in White Sponge Nevue; More prominent than

Leukoedema & doesnt go away with stretch. ; Commonly seen on buccal mucosa (or any mucosa)

Know: Cytology can be used for Pemphigus, Candidiasis, ?????, and White Sponge Nevus

Dariers Disease (Keratosis Follicularis) Aut. dominant genodermatosis characterized by a lack of cohesion among the surface epithelial cells Mutation affects the integrity of the desmosomal complex Erythematous, pruritic papules of the skin Pits and keratoses of the palms and soles Nails that feature longitudinal lines, ridges, or splits Oral lesions= Multiple normal to white flat-topped papules

o Frequently seen on the palate or alveolar mucosa Histology= Suprabasilar acantholysis (Cleft)

o Test tube rete ridgeso Central keratin plugo Corps ronds & grains

Treatment= Sunscreens and avoidance of warm environmentso Topical keratolytic agentso Systemic retinoids

My Notes: Defect in the desmosomes so you get suprabasilar cleft like pemphigus (histo) but

Dariers is not as widespread; Foul odor in summertime due to excess keratin; commonly seen on thepalate or Alveolar Mucosa; Keep in mind that this is very similar to Pemphigus but less severe;

Cobblestone mucosal appearance

Warty Dyskeratoma (Focal Acantholytic Dyskeratosis) Solitary lesion of unknown etiology Histologically similar to the lesions of Dariers disease M>F, Older patients Solitary umbilicated papule of the head and neck Oral lesions are pink or white and primarily involve keratinized mucosa Histology= Similar to the lesions of Dariers disease, but Corps ronds and grains are rare Treatment= Conservative excision

o Excellent prognosis, with no recurrencePeutz-Jeghers Syndrome

Autosomal dominant condition caused by a defect in the LKB1 gene, which codes for a serine/threoninekinase

Significance lies in the increased incidence of GI adenocarcinoma (more with Gardners syndrome,though)

Periorificial frecklingany orifice, dont go away with sun exposure Intestinal polyps with intussusception Histology= GI polyps Benign overgrowths of intestinal glandular epithelium

o Pigmented lesionso Acanthosis with elongated rete ridgeso Dendritic melanocytes containing melanin

Treatment= Monitor for intussusception and tumor developmento 2-3% of patients will develop GI adenocarcinoma

My Notes: Polyps with PJS are not Pre-Malignant, but they are with Gardners syndrome, stillhave an increased risk of colon cancer with both; Intussusception polyps get too large and catch on

waste in bowel, gets dragged down by peristalsis & can lead to obstruction

Hereditary Hemorrhagic Telangiectasia (Osler-Weber-Rendu Syndrome) Autosomal dominant condition caused by a mutation in the endoglin (HHT1) or ALK (HHT2) genes Characterized by numerous vascular hamartomas of skin and mucosa Numerous red papules which blanch upon diascopy (pressure) Epistaxis & GI Mucosal bleeding Predisposition for arteriovenous fistulas of the lung, liver, and the brain Histology= Superficial collections of thin-walled vascular channels Treatment= None necessary for mild cases

o Nasal surgery for more severe caseso Blood transfusion


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o Dental consideration= 1% of patients will develop brain abscesses, which may indicateantibiotic prophylaxis (old Natl Board Question)

My Notes: Lesions on the GI Mucosa may rupture and lead to Chronic Iron DeficiencyEhlers-Danlos Syndromes

Heterogeneous group of connective tissue disorders Probably 6 categories of this condition currently recognized Autosomal dominant, recessive, and X-linked patterns recognized Classical type (abnormal type V collagen)

o Hyperelasticity of skino Cutaneous fragilityo Papyraceous scarringMinor injury to skin heals with a crumpled cigarette paper ap--

pearance

Hypermobility typeo Soft skino Joint hyperextensibility

Vascular type (abnormal type III collagen)o Bruisingo Arterial rupture

Oral signs= Gorlin sign- can touch tip of tongue to noseo Bruising and bleeding during minor manipulation

Treatment and Prognosis= Dependant upon the type

o Vascular type may result in aortic ruptureo Accurate diagnosis is essential

My notes= rubber man; Mostly a skin issue (fragile and elastic skin); RECURRENTSUBLUXATION OF TMJ;

Tuberous Sclerosis

Uncommon autosomal dominant syndrome caused by a mutation of the TSC1 or TSC2 (most common)genes

These genes probably have a tumor suppressor function Seizure disorders and mental retardation Facial angiofibromas Ungual fibromas CNS tumors (tubers) Shagreen patches- Connective tissue lesion usually on the trunk Ash-leaf spots- Ovoid hypopigmentation on any cutaneous surface Cardiac rhabdomyomas and angiomyolipomas (usually in kidney) Oral manifestations= Enamel pitting

o Fibrous papules, primarily of the anterior gingiva Treatment=Anticonvulsant agents

o Shortened life span, secondary to CNS or kidney disease My Notes: Classic Triad= Mental Retardation, Seizure Disorders, & Angiofibromas (KNOW)

Multiple Hamartoma Syndrome (Cowden Syndrome) Rare autosomal dominant syndrome caused by a mutation of the PTENgene The gene acts as a tumor suppressor Trichilemmomas (papules around ala, mouth and ears) Acral keratosis (Warty growth on dorsum of hands) Palmoplantar keratosis Hemangiomas, xanthomas, and lipomas Females Fibrocystic disease and breast cancer, GU tumors GI polyps Multiple oral papules High arched palate Histology= Oral lesions show nonspecific fibroepithelial hyperplasia

o Trichilemmomas Diagnosis= Two of the following:

o Facial trichilemmomaso Multiple oral papuleso Acral keratosis


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Treatment= Bilateral prophylactic mastectomies(?)o Most of the other tumors are benign

My Notes: Dont have to know PTEN; be sure to KNOW TRIAD (Acral Keratosis, Tricholemomas,Multiple Oral Papules); May see GI Polyps; 1/3 of females with Cowdens get breast cancer;

Epidermolysis Bullosa Heterogeneous group of inherited blistering mucocutaneous disorders Autosomal dominant or recessive patterns, as well as an acquired form Three broad categories: Simple , Junctional, & Dystrophic Dominant dystrophic form

o Vesicles/bullae that become ulcers and erosions that heal with scarringo Gingival erythema, recession and reduction in vestibular depth

Recessive dystrophic formo Much more seriouso Secondary infectiono Mitten deformityo Microstomia

Histology= Simplex Intraepithelial cleftingo Junctional and dystrophic Subepithelial clefting (lamina lucida or lamina densa)

Treatment= None for milder forms,o Management of infections and deformities with the more severe formso

Increased incidence of cutaneous squamous cell carcinomao Oral management= Fluoride

Soft, non-cariogenic diet My Notes: Entirely Developmental (NOT AUTOIMMUNE) but there is an acquired form that isAutoimmune (ends in aquista; WONT BE ON TEST); Most kids with the junctional form die at

birth b/c all the skin sloughs off in birth canal; MITTEN DEFORMITY= scarring results in fusion of

the fingers (recessive dystrophic form); THESE PATIENTS CANT EAT B/C THE GINGIVA

SLOUGHS OFF!; Topical Fluoride is a must b/c they cant brush either; ***KNOW** Simplex--.

Intraepithelial clefting, Junctional and Dystrophic subepithelial clefting.; Genetic counseling is amust.

Pemphigus (Pemphigus Vulgaris) Autoimmune disorder characterized by antibodies directed against components of the epithelial

desmosome complex

Oral signs are often the first manifestations of the disease and the most difficult to resolve >50% present with oral lesions Ragged erosions and ulcerations Any oral mucosal surface Flaccid bullae on skin; oral blisters rarely seen Nikolskys sign (lateral pressure on normal skin causes bulla to form) Histology= Should sample lesional periphery

o Intraepithelial clefting ( due to autoimmune desmosome destruction)o Acantholysis

Immunopathologic Features= Should sample adjacent normal tissue for DIFo Intercellular immunoreactantso Positive direct immunofluorescenceo Positive indirect immunofluorescence

Treatment and Prognosis= Systemic corticosteroids, often with azathioprineo Prior to corticosteroid therapy, 50-90% mortality; Today, 5-10% mortality

My Notes: Autoimmunefatal w/o tx.; One of the conditions associated with SQUAMATIVEGINGIVITIS; Almost never see an intact oral blister b/c they are so friable; ACANTHOLYSIS=

the process by which the cells of the spinous layer tend to fall apart**;On DIF, IgG and IgC3 can

be demonstrated in the intercellular spaces in virtually 100% of affected patients; Must be alert to

possible consequences of long-term corticosteroid therapy (i.e. adrenal suppression (Cushings),

diabetes, weight gain, & increased susceptibility to infections)

Paraneoplastic Pemphigus Serious vesiculobullous disorder affecting patients with neoplastic disease, typically a lymphoreticular

malignancy (CLL and lymphoma)

Antibodies in response to the tumor probably cross react with components of the epithelial layer


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Clinically resembles a number of conditionso Erythema multiforme (Natl Board Questionhemorrhagic crusting of lips)o Pemphiguso Bullous lichen planuso Pemphigoid

Oral lesions= Hemorrhagic crusting of lips; Diffuse ulcerations Histology= Lichenoid mucositis with subepithelial or intraepithelial clefting Direct immunofluorescence-C3 and IgG granular/linear at BMZ or intercellular

o Indirect immunofluorescence-serum antibodies reactive to intercellular areas of ratbladder epithelium (better)

o GOLD STD Immunoprecipitationprecipitate out antibodies Treatment and Prognosis= Systemic corticosteroids plus azathioprine

o Topical corticosteroidso Antifungal Tx, prno Generally poor, high mortality due to sepsis or malignant progression (immunosuppressant

treatment lets neoplasm flourish)

My Notes= pathogenesis believed to be cross-reactivity b/t antibodies produced in response tothe tumor and antigens associated with the desmosomal complex and the basement membrane zone

of epitheliumCicatricial Pemphigoid

Autoimmune disease characterized by antibodies directed against one or more components of thebasement membrane

Clinically resembles pemphigus due to blister formation About 2x more common than pemphigus F>M, Avg. age 60 Desquamative gingivitis May see intact blisters intraorally **** Affects any mucosal surface; occasionally skin Scarring usually refers to conjunctival mucosa (symblepharon)should refer to ophthalmologist Histology= Must sample periphery of lesion

o Subepithelial clefting seen with light microscopic examination Immunopathologic Features= Should sample adjacent normal mucosa

o Linear deposition of immunoreactants at BMZo Positive direct immunofluorescenceo Negative indirect immunofluorescence*****

Treatment= Depends on extent of involvemento Oral only-topical corticosteroids or dapsoneo Ocular lesions require systemic immunosuppressive therapy

Prognosis= Rarely fatalo Blindness results with untreated ocular diseaseo Condition can usually be controlledo Rarely undergoes spontaneous resolution

My Notes: Positive Nickolsky Sign; 2;1 females; can treat topically depending on severity; If apatient presents with desquamative gingivitis, ask if they have itchy eyes; If eye involvement, you

have to give systemic corticosteroidsBullous Pemphigoid

Similar autoimmune condition to cicatricial pemphigoid, with a few distinct differences Positive DIF and indirect IF-BMZ Often resolves in 2-3 years M=F, 60-80 years Skin Tense bullae Oral Rare, bullae which rupture quickly Histology and Immunopathologic Features= Subepithelial clefting

o Eosinophiliao Positive direct and indirect immunofluorescence

Treatment and Prognosis= Systemic immunosuppressant therapyo Good prognosis, often with spontaneous remission in 2-3 years


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My Notes: No oral signs really, DIFFERENTIAL DIAGNOSIS B/T BULLOUS & CICATRICALPEMPHIGOID= 1( Indirect immunofluorescence, 2) Bullous can be eliminated in 1-2 yrs, 3) bullous

doesnt scar

Erythema Multiforme (EM) Acute, self-limiting ulcerative disorder Probably immune-mediated 50%-unknown; 25%-drugs; 25%-herpes EM - Spectrum of Clinical Disease

o Erythema multiforme minor - skin or mucosa onlyo Erythema multiforme major (Stevens-Johnson syndrome)- At least two mucosal sites plus skin

involvement

o Toxic epidermal necrolysis (Lyells disease) M>F; Young adults May experience prodrome Hemorrhagic crusting of lips Widespread oral ulcers with ragged margins Labial, buccal mucosa and tongue Target lesions of skin TEN

o Diffuse sloughing of the skin and mucosao

F>M; Older patients Histology= Characteristic, but not diagnostic

o Destruction of keratinocyteso Subepithelial edema

Treatment=Supportive or topical corticosteroids for mild caseso Systemic corticosteroids for EM majoro TEN managed in burn unit

Prognosis= Good for mild to moderate caseso EM major-2-10% mortalityo TEN-34% mortalityo May be recurrent-spring and fall

Immunofluorescence= nonspecific; Drug-induce form most severe; more common in males, exceptfor TEN; Minor form only skin & mucosa; EM major may cause ocular scarring (symblepharon) likecicatrical pemphigoid; Histo: nonspecific, tests done to eliminate other diseases. DIFFERENTIAL

DIAGNOSIS FOR OCULAR SCARRING CICATRICAL PEMPHIGOID, PARANEOPLASTICPEMPHIGOID, ERYTHEMA MULTIFROME

Geographic Tongue (Benign Migratory Glossitis, Erythema Migrans) (KNOW SLIDE) Common condition of unknown etiology or pathogenesis Correlation with psoriasis is not well established F>M (2:1) Transient areas of erythema (filiform papillary atrophy) with yellow-white serpentine borders Primarily affects the tongue, although any oral mucosal surface may be involved Typically asymptomatic Associated with fissured tongue Histology= Hyperparakeratosis and acanthosis

o Elongation of rete ridgeso Munro abscessesCollections of PMNs in epitheliumo Psoriasiform mucositisenlarged rete ridges, hyperkeratosis, & acute inflammation

Treatment= Reassurance of the patiento Topical corticosteroids may be used for symptomatic cases

My Notes: looks like psoriasis under microscope; Oral CDX is no good for this, it comes back asatypical cells

Lichen Planus Common chronic mucocutaneous disease Probably immune-mediated May have only skin, only oral, or both


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F>M (3:2), Adults Skin lesions-purple, polygonal, pruritic papules Oral lesions-reticular or erosive, Oral lesions usually Bilateral Reticular-interlacing white lines, buccal mucosa Erosive-ulcers with erythema and white streaks (ErosivePainful) Histology= Hyperkeratosis

o Degeneration of basal cell layero Saw-toothed rete ridgeso Band-like infiltrate of lymphocytes

Immunopathologic Features= Non-specifico Shaggy band of fibrinogen at BMZ

Treatment= 25% have superimposed candidiasis, so anti-fungal Tx may be necessary fungalo No treatment for reticularo Topical corticosteroids for erosiveo Diprolene Gel or Temovate Gel

Prognosis= Skin lesions may resolve spontaneouslyo Oral lesions persisto Malignant potential is controversial; If premalignant, risk of transformation is probably small

My Notes= may see superficial mucoceles, too!;Psoriasis

Chronic skin disease characterized by an increase in the proliferative activity of keratinocytes

Probably involves both genetic environmental factors 2nd decade Waxing and waning course with lesions improving during the summer Scalp, elbows, knees Erythematous plaques with silvery scales Oral lesions are uncommon Histology=Marked hyperparakeratosis

o Elongated rete ridgeso Perivascular inflammationo Munro abscesses

Treatment= Mild cases None necessaryo Topical corticosteroids, UV light, PUVA therapy, or systemic immunosuppressant therapyo May persist for years, even with treatment

My Notes: looks like geographic tongue; dont know cause but do know T-Lymphocytes areproducing abnormal cytokines that increase the keratin proliferation; AUSPITS SIGN= Peel off

scale & get pinpoint hemorrhage.

Lupus Erythematosus

Immunologically mediated Essentially two varieties-very different prognoses

o Systemic LEo Chronic cutaneous LE

Systemic Lupus Erythematosuso W>M (8:1), 3rd to 4th decadeo Highly variable presentationo Fever of undetermined origin (FUO), arthritis, weight loss, renal failureo Butterfly rash over malar regiono Raynauds phenomenonWhen patient is exposed to cold/stress, the hands get white &

pale, then red due to constriction of blood vesselso Oral ulcerations in 5-40%o 95% of SLE pts.have anti-nuclear antibodies against multiple antibodieso SLE has deposition of Igs (M,C,G)@ Basement membrane

Chronic Cutaneous Lupus Erythematosuso W>M (2:1), 4th decade Wo Scaly, erythematous patches on face and scalpo Exacerbated by sun exposure (remember psoriasis gets better in sun)o Resolve after months with white scarred appearanceo Oral lesions (10%) resemble erosive LP


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Histology= Lichenoid mucositiso Vacuolization of keratinocyteso Patchy PAS-positive subepithelial depositso Edema in the lamina propriao Severe or perivascular chronic inflammatory cell infiltrate

Immunopathologic Features= SLE-anti-nuclear antibodies Anti-double-stranded DNA is rather specific Anti-Sm antibody

o CCLE-abnormal lab findings are not as common ANAs only in 30% (ANAs only good as a screening tool)

My Notes: Direct IF= deposition of one or more immunoreactants (Ig M,G or C3) in a shaggy granularand at the BMZ

Treatment=o SLE

Avoid excessive sunlight Systemic corticosteroids for severe cases Anti-malarials

o CCLE Avoid excessive sunlight Topical corticosteroids

Anti-malarials for non-responsive cases Prognosis= SLE-variable

95% 5-yr. survival with treatment yr. 75% 15-yr. survival with treatment Cause of death-renal failure

o CCLE-relatively good; 5% evolve to SLE

My Notes: Be able to distinguish b/t this and Lichen PlanusDifferential diagnosis byimmunofluorescence b/c they are the same intraorally, Patchy PAS @ basement membrane,

perivascular inflammation seen in SLE not LP; If males do get it, it is worse; Lupus Cheilitis= Red

patches on lips (similar to Lichen Planus); MAY NEED TO PREMEDICATE BECAUSE OF THE

POSSIBILITY OF LIBMAN-SACKS ENDOCARDITIS

Systemic Sclerosis (Scleroderma) Rare, probably immunologically mediated deposition of collagen within the tissues While the disease most notably affects the skin, almost all organs and tissues are involved F>M; Adults Skin changes often noted first Purse string mouth Acro-osteolysis (resorption of the terminal phalanges leading to a claw appearance) Skin becomes hard with a smooth texture Mouse facies Raynauds phenomenon Dysphagia Radiograph shows diffuse widening of the PDL (also resorption of coronoid and TMJ) Histology= Dense collagen deposition

o Collagen replaces the normal tissues Diagnosis= Raynauds phenomenon and skin texture are suggestive

o Skin biopsyo Anti-Scl 70 (topoisomerase I) antibodies with anti-centromere antibodies to a lesser

extent Treatment= Difficult

o D-penicillamine, extracorporeal photochemotherapy, esophageal dilationo 80% 2-year survival, 30% (at best) 12 year survival,

My Notes= WORST PROGNOSIS OF IMMUNOLOGICAL CONDITIONS; Excess deposition ofcollagen; Raynauds will be one of the 1st signs; Pulmonary fibrosis heart failure death;Diagnosis by presence of anti-topoisomerase antibodies (anti-sl70)


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CREST Syndrome

Possibly a mild variant of systemic sclerosiso Calcinosis cutiso Raynauds phenomenono Esophageal dysfunctiono Sclerodactylyo Telangiectasia

Diagnosis and Histology=Anti-centromere antibodieso Biopsy:

Similar but milder to systemic sclerosis Telangiectasia

Treatmento Similar, but less aggressive than systemic sclerosiso Much better prognosis

My Notes: Mainly remember the acronym; Sclerodactyly= just get the claw look but not theresorption; Telangiectasia similar to HHET; Anti-Centromere antibodies (not Anti-topoisomerase);

Calcinosis cutis= movable, nontender, subcutaneous nodules

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