Oral Path Exam Iv

Front 1

Heterogeneous group of inherited (AR) metabolic disorders characterized by a lack of one or more enzymes for glycosaminoglycan synthesis: heparin sulfate, dermatan sulfate, keratin sulfate or chondroitin sulfate

Back 1

Mucopolysaccharidoses

Front 2

Common signs include:
course facial features,
mental retardation,
joint stiffness,
corneal clouding, macroglossia, gingival hyperplasia, pointed cusps on posterior teeth,
diastemas,
impacted teeth with large follicles, obliterated pulp chambers and root canals

Back 2

Mucopolysaccharidoses

Front 3

what is the prognosis of mucopolysaccharidoses?

Back 3

poor

Front 4

MPS I-VI; Hurler (I-H)
Scheie (I-S)
Hunter (II)
Sanfilippo (III-A or B)
Morquio (IV-B or V-A)
Maroteaux-Lamy (VI)

Back 4

mucopolysaccharidoses

Front 5

what sort of dental care is given to mucopolysaccharidoses patients?

Back 5

routine dental management

Front 6

-premature closure of the sagittal and metopic sutures=scaphocephaly
-sphenoid bone is hypoplastic, resulting in a "shoe" shaped sella. -frontal bossing and small facial bones.

Back 6

Hurler Syndrome (mucopolysaccharidoses)

Front 7

-Group of rare inherited disorders (AR); lack certain enzymes needed for processing some lipids (lipid storage)
-Increased incidence in Ashkenazi Jews

Back 7

Lipid Reticuloendothelioses

Front 8

Gaucher (bone marrow macrophage), Niemann-Pick (macrophage), Tay-Sachs (neurons)
are examples of?

Back 8

Lipid Reticuloendothelioses

Front 9

Which Lipid Reticuloendothelioses are treatable?

Back 9

severe not mild Gaucher (bone marrow macrophage) by enzyme replacement therapy w/ glucocerebrosidase

Front 10

An inborn error of metabolism (glucocerebrosidase deficiency).

Back 10

Gaucher Disease

Front 11

what is the gender predilection for Gauchers?

Back 11

equal

Front 12

these are the most common manifestations of which disease?
enlargement of the liver and spleen, anemia
reduced platelets
bone pain
bone infarctions often leading to damage to the shoulder or hip joints, and a generalized demineralization of the bones (osteoporosis)
spontaneous fractures.

Back 12

Gauchers

Front 13

Gauchers presents radiographically with ill defined radiolucencies in which arch most commonly?

Back 13

mandible

Front 14

Rare inherited (AR) condition characterized by the deposition of PAS+ lamellar and amorphous substance (basal lamina?) into dermis & submucosa

Back 14

Lipid Proteinosis

Front 15

Lipoid nodules in :
larynx
pharynx
esophagus
tonsils
skin
tongue

Back 15

Lipid Proteinosis

Front 16

Excess bilirubin in bloodstream

Back 16

jaundice

Front 17

Yellowish discoloration of skin and mucosa
Affinity for elastin in sclera, lingual frenum & soft palatal mucosa

Back 17

jaundice

Front 18

Heterogeneous group of disorders characterized by deposition of extracellular proteinaceous substances with β-pleated sheet molecular configuration

Back 18

amyloidosis

Front 19

the following stains are diagnostic for ?
Congo red+ (easiest most expensive)
crystal violet (metachromasia) thioflavin-T

Back 19

amyloidosis

Front 20

amyloidosis can either systemic or ____

Back 20

orgain limited

Front 21

what is the age and gender predilection of Primary and Myeloid-associated amyloidosis?

Back 21

65+
males

Front 22

the following are (early/ late) symptoms of ______
fatigue, weight loss, paresthesia, hoarseness, edema, orthostatic hypotension

Back 22

EARLY Primary and Myeloid-associated amyloidosis

Front 23

the following are (early/late) symptoms of ______
carpal tunnel syndrome, mucocutaneous nodules, hepatomegaly and macroglossia (10-40%), submucosal hemorrhage, xerostomia and jaw claudication

Back 23

LATE Primary and Myeloid-associated amyloidosis

Front 24

these pts. may respond to colchicine , steroid or melphalan therapy?

Back 24

Primary and Myeloid-associated amyloidosis

Front 25

most pts of amyloidosis die of _____ or _____ w/in months or years

Back 25

cardiac
renal

Front 26

Associated with a chronic infection or inflammatory process such as osteomyelitis, tuberculosis, sarcoidosis, bronchiectasis rheumatoid arthritis & IBD

Back 26

secondary amyloidosis

Front 27

which of the following is not involved in secondary amyloidosis?
liver
spleen
heart
kidney
adrenals

Back 27

heart

Front 28

Β-2 microglobulin (normal protein) not removed by dialysis
Especially prone to accumulate in bones & joints; tongue involvement reported
Renal transplant stops progression

Back 28

Hemodialysis-associated Amyloidosis

Front 29

Uncommon AD (Swedish, Portuguese & Japanese) or AR in cases of familial Mediterranean fever
Polyneuropathy, cardiomyopathy, arrhythmia, CHF & renal failure
May respond to colchicine therapy

Back 29

Heredofamilial Amyloidosis

Front 30

Poor vision (and blindness due to keratomalacia)
Eczema
Possible oral leukoplakia

Back 30

Vitamin A deficiency

Front 31

Beriberi most often in alcoholics/malnourished
Produces peripheral vasodilatation, edema
heart failure
peripheral neuropathy, and Wernicke-Korsakoff syndrome

Back 31

Vitamin B1(thiamin)

Front 32

if one has persistent symptom complex of ophthalmoplegia, ataxia, and an acute confusional state one would deem this ______ due to ____ deficiency

Back 32

Wernicke-Korsakoff syndrome
due to Vitamin B1(Thiamin)

Front 33

Pain
Tingling
Loss of feeling (sensation) in hands and feet
Muscle damage with loss of muscle function or paralysis of the lower legs
Vomiting
Strange eye movements (nystagmus)
Mental confusion/speech difficulties
Difficulty walking
Coma
Death

Back 33

dry berberi

Front 34

Swelling of the lower legs
Increased heart rate
Lung congestion
Enlarged heart related to congestive heart failure
Shortness of breath with activity
Awakening at night short of breath

Back 34

wet berberi

Front 35

Dermatitis (face-neck-forearms)
Dementia
Diarrhea

Back 35

pellagra

Front 36

pellagra is a symptom complex due to ____ deficiency

Back 36

vitamin B3 (niacin)

Front 37

a deficiency in ____ may cause antagonist drug (isoniazid, cycloserine, penacillamine) may produce deficiency
Produces weakness
dizziness & seizures
sometimes cheilitis and glossitis

Back 37

vitamin B6 (pyridoxine)

Front 38

Petechial hemorrhages and ecchymosis
Retarded healing
Gingival swelling and hemorrhage (scorbutic gingivitis), ulceration, tooth mobility and periodontitis

Back 38

Vitamin C deficiency

Front 39

scurvy

Back 39

vitamin C deficiency

Front 40

Back 40

amyloidoisis

Front 41

Back 41

amyloidoisis

Front 42

Back 42

amyloidoisis

Front 43

Back 43

amyloidoisis

Front 44

Back 44

amyloidoisis

Front 45

Back 45

amyloidoisis

Front 46

Back 46

jaundice

Front 47

Back 47

jaundice

Front 48

Back 48

lipid proteinosis

Front 49

Back 49

lipid proteinosis

Front 50

Back 50

lipid proteinosis

Front 51

Back 51

mucosaccharidoses

Front 52

Back 52

mucosaccharidoses

Front 53

Back 53

mucosaccharidoses

Front 54

Back 54

mucosaccharidoses

Front 55

Back 55

mucosaccharidoses claw hand

Front 56

Back 56

mucosaccharidoses corneal clouding

Front 57

Irritability
Growth retardation
Costochondral prominence (rachitic rosary)
Enamel hypoplasia, dentin disturbance, & delayed eruption
characterizes the condition of ____ due to the deficiency of ______

Back 57

childhood rickets
vitamin D

Front 58

the following are symptoms of which form of Vit D deficiency?
Osteopenia
Fracture
Skeletal pain
?Periodontitis

Back 58

Adult Osteomalacia

Front 59

rare and usually associated with poor fat absorption
Neurological abnormalities inconsistently reported
Associated oral problems not known

Back 59

Vitamin E deficiency

Front 60

______ deficiency associated with severe fat malabsorption syndromes, antibiotic use or dicumarol therapy
Results in hypoprothrombinemia, ecchymosis & hemorrhage

Back 60

vitamin K

Front 61

it is necessary to check pts _____ when on anticoagulation therapy for Vit K deficiency for dental procedures

Back 61

prothrombin time

Front 62

what is the most common form of anemia in the USA?

Back 62

iron deficiency

Front 63

Fatigue, palpitations, lightheadedness, angular cheilitis, atrophic glossitis, mucosa atrophy, tenderness or burning tongue, predisposition to oral candidiasis may be a result of ______ deficiency?

Back 63

iron deficiency

Front 64

Also known as the Paterson-Kelly syndrome or sideropenic dysphagia

Back 64

Plummer-Vinson Syndrome

Front 65

Fatigue, weakness & SOB
Chronic iron deficiency anemia, glossitis & dysphagia
Esophegeal webs and koilonychia (spoon-shaped nails)
Scandinavian or Northern European Women, 30-50 years old

Back 65

Plummer-Vinson Syndrome

Front 66

Glossopyrosis, glossodynia & angular cheilitis
Considered premalignant due to high incidence of oral, hypopharyngeal and esophageal carcinoma

Back 66

Plummer-Vinson Syndrome

Front 67

Poor absorption of vitamin B12 (cobalamin) in duodenum due to lack of intrinsic factor (from parietal cells of stomach); needed for nucleic acid synthesis—retarded RBC formation & maturation produces megaloblastic anemia and atrophy of lining of GI tract

Back 67

pernicious anemia

Front 68

Burning of lips, tongue (50-60%), buccal mucosa & other sites; erythema and trophy of mucosa
Increased risk of gastric carcinoma (est. 1-2%)
Cobalamin (B12) injection or high dose oral cobalamin clears oral lesions in a few days

Back 68

pernicious anemia

Front 69

Rare condition with low levels of growth hormone or defective tissue response
Proportional but stunted growth
Some show both delayed tooth eruption and exfoliation for one to several years; often lack third molars
Treatment with growth hormone prior to epiphyseal closure

Back 69

pituitary dwarfism

Front 70

Rare condition of increased growth hormone usually from pituitary adenoma
Accelerated proportional growth (>3 sd) prior to epiphyseal closure; acromegaly after epiphyseal closure

Back 70

gigantism

Front 71

gigantism is associated with ________ syndrome

Back 71

McCune Albright

Front 72

Rare condition of increased growth hormone after epiphyseal closure
Usually from pituitary adenoma; est. 66 cases/million; average age is 42 at diagnosis.

Back 72

acromegaly

Front 73

Headache, visual disturbances and other hormonal abnormalities
Enlarged hands, feet, cranium, mandible (prognathism, apertognathia & diastema), soft palate (sleep apnea), tongue (macroglossia)

Back 73

acromegaly

Front 74

Back 74

acromegaly

Front 75

Back 75

acromegaly

Front 76

Back 76

acromegaly

Front 77

Back 77

acromegaly

Front 78

Back 78

acromegaly

Front 79

Back 79

acromegaly

Front 80

Back 80

acromegaly

Front 81

Back 81

acromegaly

Front 82

Back 82

acromegaly

Front 83

Back 83

acromegaly

Front 84

Back 84

gigantism

Front 85

Back 85

pellagra

Front 86

Back 86

acromegaly

Front 87

Back 87

acromegaly

Front 88

Back 88

acromegaly

Front 89

Back 89

pellagra necklace

Front 90

Back 90

pellagra necklace

Front 91

Back 91

pernicious anemia

Front 92

Back 92

pernicious anemia

Front 93

Back 93

pernicious anemia

Front 94

Back 94

plummer vinson syndrome atrophic glossitis

Front 95

Back 95

esophageal webs in plummer vinson syndrome

Front 96

Back 96

koilonychia of plummer vinson syndrome

Front 97

Back 97

rickets

Front 98

Back 98

rickets

Front 99

Back 99

scurvy

Front 100

Back 100

scurvy

Front 101

Back 101

corkscrew hair of scurvy

Front 102

Back 102

gingival hemorrhage of scurvy

Front 103

Back 103

periungual hemorrhage of scurvy

Front 104

Back 104

adult osteomalacia due to vit D deficiency

Front 105

Back 105

vitamin A follicular keratosis

Front 106

Back 106

vitamin A oral leukoplakia

Front 107

Back 107

vitamin A oral leukoplakia

Front 108

Back 108

Vitamin B neuronal degeneration

Front 109

Back 109

vitamin K deficiency

Front 110

Back 110

vitamin K deficiency

Front 111

Due to decreased levels of thyroid hormone (thyroxine); primary or secondary (pituitary TSH decreased)
Deposition of glycosaminoglycan ground substance—non-pitting edema

Back 111

Hypothyroidism

Front 112

What is the hypothyroid state referred to in :

infancy?
adulthood?

Back 112

cretinism
myxedema

Front 113

Lethargy, dry course skin, facial swelling, non-pitting peripheral edema, huskiness of voice, constipation, weakness, fatigue, bradycardia, hypothermia, hypotension
Enlargement of lips and tongue

Back 113

Hypothyroidism

Front 114

how might hypothyroidism affect teeth

Back 114

delayed eruption and exfoliation

Front 115

Excess production of thyroid hormone (thyroxine)

Back 115

hyperthyroidism

Front 116

what is the most common cause of hyperthryroidism?

Back 116

Graves disease (60-90%)

Front 117

autoantibodies to TSH receptors attach and increase thyroxine release

Back 117

graves disease

Front 118

what is the gender predilection of hyperthyroidism?

Back 118

females 5x-10x

Front 119

Nervousness, heart palpitations, heat intolerance (excessive perspiration), emotional lability, weakness, fatigue, weight loss ( despite increased appetite), tachycardia, increased systolic/decreased diastolic pressure, fine tremor, exophthalmos or proptosis (20-40%)

Back 119

hyperthyroidism

Front 120

Increased sensitivity to native and supplemental epinephrine and exaggerate tachycardia and hypertension

Back 120

hyperthyroidism

Front 121

Reduced amount of PTH (parathyroid hormone) leading to calcium imbalance

Back 121

hypoparathyroidism

Front 122

what pathology is associated with the removal during thyroidectomy, autoimmune destruction,
DiGeorge syndrome
endocrine-candidiasis syndrome

Back 122

hypoparathyroidism

Front 123

the following are symptoms of what?
-Hypocalcemia
-Chvostek’s sign (twitching of upper lip by tapping facial nerve just below zygomatic process)
-enamel hypoplasia if present during odontogenesis
-oral candidiasis

Back 123

hypoparathyroidism

Front 124

Rare group of conditions (AD or sporadic) in which target cells fail to properly respond to PTH

Back 124

Pseudohypoparathyroidism

Front 125

Pseudohypothyroidism types
-type 1a?
-type 1b?
-type 1c?
-type 2?

Back 125

type1a: defect in binding protein activation

type 1b: defective receptor

type 1c: reason unclear

type 2: normal protein and binding, defective functional response

Front 126

Tooth abnormalities include enamel hypoplasia, widened pulp chambers, “dagger-shaped” pulp calcifications, oligodontia, delayed eruption & blunt apices

Back 126

pseudoparathyroidism

Front 127

Excess production of parathyroid hormone

Back 127

hyperparathyroidism

Front 128

what is the most common cause of primary hyperparathyroidism, and the 2nd and 3rd?

Back 128

1)parathyroid adenoma 80-90%
2) parathyroid hyperplasia
3) parathyroid carcinoma

Front 129

secondary hyperparathyroidism is often associated with ?

Back 129

end stage renal failure due to lack of Vit of D

Front 130

both primary and secondary hyperparathyroidism are associated with a tumor that resembles __?

Back 130

central giant cell granuloma of the jaws

Front 131

what is the tag for hyperparathyroidism?

Back 131

stones, bones, stomach groans and psychic moans

Front 132

"stone" of hyperparathyroidism = ?

Back 132

nephrolithiasis
metastatic calcifications

Front 133

"bones" of hyperparathyroidism =?

Back 133

loss of lamina dura
subperiosteal resorption in phalanges
ground glass appearance of bone
brown tumors of bone

Front 134

"stomach groans" of hyperparathyroidism = ?

Back 134

duodenal uclers

Front 135

"psychic moans" of hyperparathyroidism?

Back 135

depression, lethargy, weakness, seizures, confusion & dementia

Front 136

which of the following occur in primary or secondary hyperparthyroidism?
Hypercalcemia ____ hypophosphatemia _____ hyperphosphatemia _____
hypercalciuria & hyperphosphaturia _____

Back 136

Hypercalcemia (both), hypophosphatemia (primary), hyperphosphatemia (secondary), hypercalciuria & hyperphosphaturia (both)

Front 137

Back 137

goiter

Front 138

Back 138

goiter

Front 139

Back 139

hyperparathyroidism

Front 140

Back 140

hyperparthyroidism

Front 141

Back 141

brown tumor of hyperparathyroidism

Front 142

Back 142

ground glass bone of hyperparathyroidism

Front 143

Back 143

ground glass bone of hyperparathyroidism

Front 144

Back 144

ground glass bone of hyperparathyroidism

Front 145

Back 145

secondary hyperparathyroidism

Front 146

Back 146

secondary hyperparathyroidism

Front 147

Back 147

secondary hyperparathyroidism

Front 148

Back 148

hyperparathryoidism ground glass appearance

Front 149

Back 149

hyperparathyroidism

Front 150

Back 150

enamel hypoplasia of hypoparathyroidism

Front 151

Back 151

enamel hypoplasia of hypoparathyroidism

Front 152

Back 152

enamel hypoplasia of hypoparathyroidism

Front 153

Back 153

hypothryoidism

Front 154

Back 154

hypothryoidism

Front 155

Back 155

hypothryoidism

Front 156

Back 156

hypothryoidism

Front 157

Back 157

hypothryoidism

Front 158

Back 158

hypothryoidism

Front 159

Back 159

hypothryoidism before and after

Front 160

Back 160

hypothryoidism before and after

Front 161

Back 161

pseudoparathyroidism

Front 162

Back 162

pseudoparathyroidism

Front 163

Back 163

pseudoparathyroidism

Front 164

Back 164

pseudoparathyroidism

Front 165

Back 165

pseudoparathyroidism

Front 166

what is the most common form of hypercortisolism?

Back 166

Cushing Syndrome

Front 167

a result of a high level of exogenous glucocorticoids?

Back 167

Cushing Syndrome

Front 168

a result of endogenous causes such as excess ACTH secretion by pituitary

Back 168

Cushings Disease

Front 169

symptoms:
Weight gain (central), “buffalo hump”, “moon facies” red-purple abdominal striae, hirsutism, poor healing, osteoporosis hypertension, depression, hyperglycemia, polydypsia, polyuria muscle wasting/weakness

Back 169

Hypercortisolism

Front 170

Insufficient production of adrenal corticosteroid hormone

Back 170

Addison's disease

Front 171

Addisons caused by the following are those of primary or secondary nature?
-autoimmune destruction
-infection; TB & deep fungal (AIDS) -metastasis
-sarcoidosis
-lack of ACTH
-hemochromatosis
-amyloidosis

Back 171

primary:
-autoimmune destruction
-infection; TB & deep fungal (AIDS) -metastasis
-sarcoidosis
-hemochromatosis
-amyloidosis
Secondary:
-lack of ACTH

Front 172

Onset of ____ @>90% destruction fatigue, irritability, depression, weakness, hypotension, oral freckling first followed by skin hyperpigmentation of sun-exposed and over pressure points (ACTC or β-lipotropin), GI upset( anorexia, nausea, diarrhea, vomiting) and weight loss & salt craving

Back 172

Addison's disease

Front 173

Common disorder of carbohydrate metabolism, 5-10% type I-decreased production (autoimmune) or 90-95% type II-tissue resistance

Back 173

diabetes mellitus

Front 174

Main complications related to peripheral vascular disease and impaired neutrophil function (atherosclerosis, IHD, ABI, renal failure, retinopathy, gangrene-amputation)

Back 174

diabetes mellitus

Front 175

Increased incidence & severity of periodontitis, delayed healing, oral candidiasis, diabetic sialadenosis, zygomycosis,  benign migratory glossitis, xerostomia

Back 175

diabetes mellitus

Front 176

Back 176

addisons

Front 177

Back 177

addisons

Front 178

Back 178

addisons

Front 179

Back 179

addisons

Front 180

Back 180

addisons

Front 181

Back 181

addisons

Front 182

Back 182

cushing syndrome

Front 183

Back 183

cushing syndrome

Front 184

Back 184

ab striations of cushing syndrome

Front 185

Back 185

diabetes mellitus

Front 186

Back 186

diabetes mellitus

Front 187

Back 187

diabetes mellitus

Front 188

Back 188

diabetes mellitus

Front 189

Rare group of metabolic bone diseases with deficiency of tissue-nonspecific alkaline phosphatase; most forms are autosomal; perinatal, infantile, childhood & adult forms

Back 189

Hypophosphatasia

Front 190

-Bone abnormalities resemble rickets
-reduced serum alkaline phosphatase & increased serum & urinary phosphoethanolamine levels
-Poorly mineralized osteiod or poor maturation of bone, bowing and fracture of bones

Back 190

Hypophosphatasia

Front 191

how might hypophosphatasia affect the dentition

Back 191

premature exfoliation
permanent have little cementum
hypoplasia of alveolar bone

Front 192

Rare, usually X-linked dominant (1/20,000), also known as hereditary hypophosphatemia caused by mutation of gene for zinc metalloproteinase PHEX (phosphate regulating gene with endopeptidase activity on the X chromosome) resulting in an inability to convert vitamin D into active form

Back 192

Hypophosphatemia (Vit D-resistant Rickets)

Front 193

Results in low phosphorus and calcium level and a rickets-like state

Back 193

Hypophosphatemia

Front 194

hypophosphatemia may be treated with ____

Back 194

calcitrol (activeVit D supplement)

Front 195

Teeth typically have large pulp chambers, sometimes with clefts in dentin/enamel, most have pulp horns that approximate the DEJ with risk of pulp exposure with attrition of enamel (25+% with multiple abscesses)

Back 195

hypophosphatemia

Front 196

Inflammatory disease of GI tract, especially distal small intestine & proximal large bowel (regional ileitis/enteritis)

Back 196

Crohns Disease

Front 197

a teenager w/ symptoms of abdominal cramps & pain, nausea, diarrhea, anemia malnourishment

Back 197

Crohns Disease

Front 198

oral lesions reported from Crohns Disease occurs about ___% of time

Back 198

30%

Front 199

-Oral lesions vary from diffuse or nodular oral/perioral swellings, cobblestone appearance of mucosa, mucosal ulcers and fissures or present as pyostomatitis vegetans
-Biopsy of oral lesions can show non-necrotizing granulomatous inflammation

Back 199

Crohns Disease

Front 200

what Type of medications are used for treating Crohns Disease?
cortisol?
analgesics?
antibiotics?

Back 200

antibiotics

Front 201

Rare manifestation of ulcerative colitis or Crohn’s disease; can precede or be concurrent
Yellowish, slightly elevated linear, serpentine pustules on an erythematous mucosa (“snail-tract ”ulcerations)

Back 201

Pyostomatitis Vegetans

Front 202

where will pyostomatitis vegetans often occur in the oral cavity?

Back 202

buccal
labial soft palate mucosa
tongue

Front 203

how is Pyostomatitis Vegetans treated?

Back 203

antibiotics

Front 204

White plaques (damaged mucosa) on buccal mucosa, tongue & floor of mouth; unpleasant taste, oral pain & burning and ammonia breath and is associated uncommonly with renal failure

Back 204

uremic stomatitis

Front 205

Back 205

crohns disease

Front 206

Back 206

crohns disease

Front 207

Back 207

crohns disease

Front 208

Back 208

crohns disease

Front 209

Back 209

crohns disease

Front 210

Back 210

crohns disease

Front 211

Back 211

crohns disease

Front 212

Back 212

hypophosphatasia

Front 213

Back 213

hypophosphatasia

Front 214

Back 214

pyostomatitis vegetans

Front 215

Back 215

pyostomatitis vegetans

Front 216

Back 216

pyostomatitis vegetans

Front 217

Back 217

pyostomatitis vegetans

Front 218

Back 218

pyostomatitis vegetans

Front 219

Back 219

uremic stomatitis

Front 220

Back 220

uremic stomatitis

Front 221

Back 221

Vit D rickets

Front 222

Back 222

vit D rickets

Front 223

Back 223

vit D rickets

Front 224

Back 224

vit D rickets

Front 225

Back 225

vit D rickets

Front 226

Back 226

vit D rickets

Front 227

Back 227

vit D rickets

Front 228

Back 228

vit D rickets

Front 229

A group of inherited conditions in which two or more ectodermally derived structures fail to develop.

Back 229

ectodermal dysplasia

Front 230

Patients usually display heat intolerance due to a reduced number of sweat glands; diagnosis may be made in infancy due to a “fever of unknown origin”; rare deaths due to markedly elevated body temperature.

Back 230

hypohidrotic ectodermal dysplasia

Front 231

what is the gender predilection for hypohidrotic ectodermal dysplasia

Back 231

males

Front 232

how will ectodermal dysplasia affect the teeth?

Back 232

oligodontia
hypodontia
pointed/tapered crowns

Front 233

The dental treatment of ectodermal dysplasia is usually accomplished by _____ replacement

Back 233

prosthetic

Front 234

Cannon’s Disease and Familial White Folded Dysplasia.

Back 234

White Sponge Nevus

Front 235

Condition due to defect in the normal keratinization of the mucosa. Related to keratin 4 and keratin 13 with mutations of either of the genes responsible for these keratins are known to cause the clinical manifestations

Back 235

White Sponge Nevus

Front 236

appears as symmetric,thickened, white corrugated/velvety plaque

Back 236

White Sponge Nevus

Front 237

the White Sponge Nevus affects what oral aspect most often?

Back 237

buccal mucosa

Front 238

what is the necessary tx for a White Sponge Nevus?

Back 238

none necessary

Front 239

Rare autosomal recessive trait caused by several defects in excision repair and/or postreplication repair mechanism of DNA.
Result is inability of epithelium to repair UV- light-damage.

Back 239

Xeroderma Pigmentosum

Front 240

-tendency to sunburn w/ skin atrophy
-pigmentation and depigmentation occur

Back 240

Xeroderma Pigmentosum

Front 241

the risk of _______ may lead to Childhood actinic keratoses develop with squamous cell, basal cell or melanocarcinomas developing at ages earlier than usual.

Back 241

Xeroderma Pigmentosum

Front 242

Eighty percent of the patients have subnormal intelligence.
Oral manifestations: Often squamous cell carcinoma develops on the lower lip and tip of tongue before age 20.

Back 242

Xeroderma Pigmentosum

Front 243

what is the prognosis of Xeroderma Pigmentosum?

Back 243

not good.
death at early age

Front 244

A mutant gene that encodes an intracellular calcium pump has been identified as the cause for the abnormal desmosomal organization.

Back 244

Darier's Disease

Front 245

Numerous erythematous, pruritic papules on trunk and scalp develop during second decade; lesions are rough and degradation of the accumulated keratin gives a foul odor.
Palms and soles may exhibit pits and keratosis.
The nails may show lines, ridges or painful splits.

Back 245

Darier's Disease

Front 246

In Darier's disease oral lesions are numerous normal colored/or white, flat-topped papules in a cobblestone presentation, most often on which 2 sites?

Back 246

hard palate
alveolar mucosa

Front 247

dyskeratotic process characterized by a central keratin plug which overlies epithelium exhibiting a suprabasilar cleft but not limited to_______

Back 247

Darier's Diesease

Front 248

-The intraepithelial clefting is known as acantholysis
-The rete pegs
- Dyskeratotic cells (corps ronds or grains)

Back 248

Darier's Disease

Front 249

Back 249

white sponge nevus

Front 250

Back 250

white sponge nevus

Front 251

Back 251

xeroderma pigmentosum

Front 252

Darier's disease is a premalignant condition (T/F)?

Back 252

F

Front 253

Uncommon solitary lesion that occurs on either the skin or oral mucosa. It is histologically identical to Darier’s disease

Back 253

Warty Dyskeratoma

Front 254

what is the predilections for Warty Dyskeratoma?

Back 254

Males
>40

Front 255

-looks like Darier's Disease
-Intraorally, it appears as a pink to white, umbilicated papule located on keratinized mucosa especially the alveolar ridge and palate.

Back 255

Warty Dyskeratoma

Front 256

-Autosomal dominant trait with up to 35% new mutations.
-A gene known as STK11 (also known as LKB1), which encodes for a serine/threonine kinase, is considered responsible for this syndrome.
-Characterized by freckle-like lesions of hands, perioral, perinasal skin and oral mucosa.
-Patients manifest intestinal polyposis.

Back 256

Peutz-Jeghers Syndrome

Front 257

skin lesions of Peutz-Jeghers Syndrome often appear in what age group ____ and on what features commonly ____?

Back 257

childhood
extremities (50%)

Front 258

Intestinal polyps are considered _____ and most commonly affect _____ and ______ intussusception, obstruction are common bowel problems of P-J syndrome

Back 258

hamartomas
ileum and jejunum

Front 259

P-J syndrome:
- 2-3% develop GI ____
- oral blue-gray-brown macules develop 90% of the time on labial/buccal mucosa and ____

Back 259

adenocarcinomas
tongue

Front 260

Olser-Weber-Rendu Syndrome

Back 260

HEREDITARY HEMORRHAGIC TELANGIECTASIA (HHT)

Front 261

- Mutation of either one of two different genes is responsible for this condition; endoglin gene (HHT1) or activin receptor-like kinase-1 gene (HHT2).
- Numerous vascular problems such as vascular and A-V fistulas arise.

Back 261

HEREDITARY HEMORRHAGIC TELANGIECTASIA (HHT)

Front 262

what symptom is often first presented upon seeing a HHT patient?

Back 262

epistaxis
- due to numerous red papules on naso-oropharyngeal mucosa

Front 263

what are the 3 common areas for HHT to present within the mouth?

Back 263

1) lips
2) tongue
3) buccal mucosa

Front 264

CREST sydrome and autoantibodies are differential diagnoses for what ____

Back 264

HHT

Front 265

superficial collections of thin-walled vascular spaces containing red blood cells =

Back 265

telangiectasias

Front 266

tx of HHT:
Mild cases may require no treatment.
Moderate case may be managed by selective cryosurgery or electrosurgery.
Severe cases may require ______

Back 266

septal dermoplasty.

Front 267

A group of at least 10 inherited connective tissue disorders attributed to abnormal collagen formation.
Patients usually exhibit hypermobility of joints, are easily bruised and have marked elasticity of skin.
Some patients have appeared in the circus as “contortionists”.

Back 267

Ehlers-Danlos Syndrome

Front 268

the classical and classical (mild) types of E-D syndromes involve what type of collagen defect?

Back 268

collagen V

Front 269

Kyphoscoliosis, Arthrochalasis, and Dermatosparaxis are all variations of what syndromes?

Back 269

Ehlers-Danlos Syndrome

Front 270

what is the Gorlin Sign?

Back 270

pts that can touch the tip of their nose with tongue. E-D syndromes (50%)

Front 271

-Minor manipulations of oral tissue may cause bruising and/or bleeding.
-TMJ subluxation has been reported.
-Tooth abnormalities not an usual component but malformed, hypoplastic teeth have been reported for what ?

Back 271

Ehlers Danlos Syndromes

Front 272

what type of E-D syndrome is a concern for rupturing of the aorta?

Back 272

ecchymotic type

Front 273

Uncommon syndrome characterized by seizure disorders, mental retardation and angiofibromas of the skin.

Back 273

Tuberous Sclerosis

Front 274

Epiloia
Bourneville-Pringle Syndrome is also know as?

Back 274

Tuberous Sclerosis

Front 275

Oral manifestations include:
-50-100% have pitting of enamel of anterior permanent dentition.
-11-56% have fibrous papules most commonly on anterior gingiva.
Diffuse gingival fibrous enlargement has been reported but most in patients who are taking phenytoin.

Back 275

Tuberous Sclerosis

Front 276

Cowden Syndrome; PTEN Hamartoma-Tumor Syndrome is commonly referred to as _____

Back 276

Multiple Hamartoma Syndrome

Front 277

A mutation of the PTEN (phosphatase and tensin homolog deleted on chromosome 10) gene has been implicated

Back 277

Multiple Hamartoma Syndrome (Cowden Syndrome)

Front 278

The skin lesions often appear as small papules primarily on the facial skin around the mouth, nose and ears. These papules are hair follicle hamartomas known as trichilemmomas. Other prominent skin lesions include: acral keratosis, palmoplantar keratosis, hemangiomas, xanthomas and lipomas.

Back 278

Multiple Hamartoma Syndrome (Cowden Syndrome)

Front 279

Oral lesions of ______ include:
Oral lesions typically consist of multiple papules affecting the gingivae, dorsal tongue and buccal mucosa. High-arched palate, periodontitis and extensive dental caries has also been reported.

Back 279

Multiple Hamartoma Syndrome (Cowden Syndrome)

Front 280

Diagnosis is based on finding two of the following three signs:
1) multiple facial trichilemmomas
2) multiple oral papules
3)

Back 280

3) acral keratoses. A family history is also helpful.

Front 281

Describes a group of inherited blistering mucocutaneous disorders which have in common defects in the attachment mechanisms of the epithelial cells

Back 281

EPIDERMOLYSIS BULLOSA

Front 282

The 4 broad categories of Epidermolysis Bullosa are:
1) Simplex
2)
3) Dystrophic
4)

Back 282

2) Junctional
4) Hemidesmosomal

Front 283

Epidermalysis Bullosa:
-Simplex type is a defect in ____
-Junction type is a defect in ___
-Dystrophic type is a defect in ___

Back 283

Simplex - Keratin
Junctional - Hemidesmisomes
Dystrophic - type VII collagen

Front 284

what type of Epidermolysis predisposes one to SCC?

Back 284

dystrophic type

Front 285

what type of Chronic Vesiculoulcerative Disease is the only one with a predilection for males,
is non-diagnostic for immunofluorescence and negative for indirect immunofluorescence

Back 285

Erythema Multiforme

Front 286

what is the most common type of Pemphigous autoimmune disorder?

Back 286

P. vulgaris

Front 287

The blistering that is seen with this disease results from autoantibodies directed against epidermal cell surface glycoprotein a component of desmosomes (desmoglein 3 and desmoglein 1)
- most often displayed in the oral cavity first

Back 287

Pemphigus

Front 288

The average age is 50 years with the sex predilection being equal.
Vesicles, erosions and ulcerations may occur on any mucosal or skin surface.
Patients usually complain of pain or soreness with the palate, labial/buccal mucosa, ventral tongue and gingiva the more frequent sites of involvement.

Back 288

Pemphigus

Front 289

epithelial sloughing described as "row of tomstones)

Back 289

Pemphigus

Front 290

The separation of the individual epithelial cells is termed ______. The loose cells tend to become rounded ( ___ cells).

Back 290

-acantholysis
-Tzanck

Front 291

what type of immunofluroscence is typically diagnositic in the general pathology of Pemphigus?

Back 291

direct immunofluroescence

Front 292

Mucous Membrane Pemphigoid is also know as ____

Back 292

Cicatricial Pemphigoid

Front 293

This is a chronic, blistering, mucocutaneous, autoimmune disease, which clinically may resemble pemphigus hence “-oid” suffix; however prognosis and histology are different and may lead to scarring and conjuctival blindness

Back 293

Mucous Membrane Pemphigoid

Front 294

what is the gender predilection for Cicatricial/Mucous Membrane Pemphigoid?

Back 294

Females ~ 60

Front 295

Oral lesions of Mucous Membrane Pemphigoid begin as vesicles/bullae, which eventually rupture leaving large ulcers; the ulcers are painful and may persist for weeks to months without treatment; gingival lesions alone are part of a group of lesions known as _____

Back 295

desquamative gingivitis

Front 296

Ocular involvement occurs in ___ of MM Pemphigus pts, and may include:
-symblepharons (adhesions), entropion (scar turns eyelid in), trichiasis (eyelashes rub cornea and globe); eyes are dry due to closing of lacrimal duct orifices.

Back 296

25%

Front 297

The perilesional mucosa shows subepithelial clefting with mild chronic inflammatory infiltrate in connective tissue in what type of Pemphigus?

Back 297

Cicatricial/MM

Front 298

how can Cicatricial pemphigus be differentiated from Linear IgA disease?
and from Epidermolysis Bullosa?

Back 298

-IgG / C3 present for pemphigus
-IgG in ROOF of bullae, not floor as in EB

Front 299

what tx might control most of the oral manifestations of Cicatricial pemphigus and some of the systemic complications?

Back 299

Corticosteroids

Front 300

Is a vesiculobullous, ulcerative condition of uncertain etiology and pathogenesis.

Back 300

Erythema Multiforme

Front 301

50% of cases are preceded by infection such as ____or ______ one of a variety of drugs

Back 301

Herpes simplex
Mycoplasma pneumoniae

Front 302

evidence of severe, diffuse sloughing and ulceration of the entire skin and mucosal surfaces (toxic epidermal necrolysis or Lyell’s disease).

Back 302

erythema multiforme

Front 303

Prodromal symptoms include: fever, malaise, headache, cough and sore throat occurring approximately 1 week before.
-Mild form usually lasts from 2-6 wks. However, about 20% of the patients have recurrences often in spring or fall.

Back 303

Erythema Multiforme

Front 304

Early skin lesions are usually flat, round and dark red. They are more common where?_____

These lesions may evolve into bullae and develop ________

Back 304

- extremities: arms/legs
- necrotic centers

Front 305

Target (bull’s eye) skin lesions

Back 305

Erythema Multiforme

Front 306

Oral lesions begin as erythematous patches, become necrotic and form large shallow ulcers with irregular borders; ulcerative lesions of the lips may crust.
-Ulcers have a diffuse distribution but ____ and _____may be spared.

Back 306

gingiva
hard palate

Front 307

Erytthema Multiforme Major is a more sever form of erythema multiforme and is usually triggered by _____

Back 307

drugs

Front 308

____ and ____ lesions must accompany oral and skin lesions for a diagnosis of Stevens-Johnson Syndrome to be made.

Back 308

ocular and genital

Front 309

what is the most severe form of erythema multiforme?

Back 309

Toxic Erythema Necrolysis

Front 310

what is the common cause of Toxic Erythema Necrolysis and and its gender/age predilection?

Back 310

drugs
older-females

Front 311

what are the main medications for minor and major erythema multiforme conditions alike?

Back 311

corticosteroids

Front 312

Geographic tongue; benign migratory glossitis; wandering rash of the tongue = ?

Back 312

Erythema Migrans

Front 313

what is the gender predilection for Erythema Migrans?

Back 313

females

Front 314

White annular lesions with atrophic red centers; erythema (red center) is due to atrophy of filiform papillae; pattern migrates over the dorsum of the tongue; varies in intensity and may spontaneously disappear; usually asymptomatic, occasionally painful-hot or spicy foods; patients may also have fissured tongue as these two entities are often seen together.

Back 314

Erythema Migrans

Front 315

how is erythema migrans treated?

Back 315

usually left to heal

Front 316

Is erythema migrans considered to be premalignant?

Back 316

no. it is benign

Front 317

Abnormal immunologic response to infectious agent; genetic influence, presence of HLA-B27
- often presenting with clinical triad of :
-arthritis
-urethritis
-conjuctivitis
- usually with skin lesions also:
-well circumscribed erythematous erosions and apthous-like oral lesions

Back 317

Reactive Arthritis = Reiter's Syndrome

Front 318

Reactive Arthritis = ?

Back 318

Reiter's Syndrome

Front 319

Usually tx is not necessary for mild cases of Reiter's syndrome, but for urethritis ____ are generally used and ___ for arthritis. while in the end 10-25% of pts. have _____

Back 319

antibiotics: doxycycline/minocycline
NSAIDS
disabling arthritis

Front 320

A relatively common, chronic dermatologic disease, often affecting the oral mucosa; current evidence indicates it is an immunologically mediated disorder; the relationship to stress is controversial

Back 320

lichen planus

Front 321

what is the gender predilection for lichen planus?

Back 321

females 3:2

Front 322

- Skin lesions appear as pruritic, purple, polygonal papules with a fine,
- lace-like network of white lines known as Wickham’s striae; oral and other mucous membranes may be involved as well as the nails.

Back 322

lichen planus

Front 323

which of the following statemets concerning Lichen planus is false:
- a rare chronic dermatologic disease that often affects the oral mucosa
-immunologically mediated disease.
-relatively insignificant associations to hepatitis C infection

Back 323

lichen planus is a rather COMMON chronic disease

Front 324

what are the 2 forms of lichen planus?

Back 324

1) Reticular Lichen Planus
2) Erosive Lichen Planus

Front 325

what is the the most common form of lichen planus?

Back 325

Reticular

Front 326

what type of lichen planus:
It is usually asymptomatic and occurs most frequently on buccal mucosa bilaterally; may also affect the tongue, gingivae and palate.
So named because of interlacing white lines but may also appear as plaques.

Back 326

Reticular

Front 327

what type of lichen planus is usually symptomatic?

Back 327

Erosive

Front 328

form of lichen planus:
Lesions appear atrophic, erythematous with central areas of ulceration; usually the fine, white radiating striae can be seen at edge of lesion; with gingival involvement

Back 328

Erosive

Front 329

what form of lichen planus = is part of a group of specific disease entities which produce a reaction pattern called desquamative gingivitis.

Back 329

Erosive LP

Front 330

Ortho/Parakeratosis seen with ______ form of lichen planus; the spinous layer may be atrophic or hyperplastic with “saw-toothed” rete ridges; the basal layer may show hydropic degeneration.

Back 330

Reticular form

Front 331

the following describes the histological features of?
A band-like infiltrate of predominantly T lymphocytes adjacent to the epithelium is usually present.
Degenerating keratinocytes (colloid, cytoid, hyaline or Civatte bodies) may be seen at interface of epithelium and connective tissue.

Back 331

Lichen Planus

Front 332

which form of lichen planus requires a biopsy for differential diagnosis?

Back 332

Erosive Form

Front 333

Erosive lichen planus, that is symptomatic is treated with?

Back 333

corticosteroids

Front 334

Back 334

peutz-jeghers syndrome

Front 335

Back 335

peutz-jeghers syndrome

Front 336

Back 336

peutz-jeghers syndrome

Front 337

Back 337

HHT

Front 338

Back 338

Ehlers-Danlos Syndrome

Front 339

Back 339

Ehlers-Danlos Syndrome

Front 340

Back 340

Ehlers-Danlos Syndrome

Front 341

Back 341

tuberous sclerosis

Front 342

Back 342

tuberous sclerosis

Front 343

Back 343

tuberous sclerosis

Front 344

Back 344

multiple hamartoma syndrome

Front 345

Back 345

multiple hamartoma syndrome

Front 346

Back 346

epidermolysis bullosa

Front 347

Back 347

epidermolysis bullosa

Front 348

Back 348

epidermolysis bullosa

Front 349

Back 349

epidermolysis bullosa

Front 350

Back 350

pemphigus

Front 351

Back 351

pemphigus

Front 352

Back 352

pemphigus

Front 353

Back 353

Cicatricial / Mucous Membrane Pemphigus

Front 354

Back 354

Cicatricial / Mucous Membrane Pemphigus

Front 355

Back 355

Cicatricial / Mucous Membrane Pemphigus

Front 356

Back 356

Cicatricial / Mucous Membrane Pemphigus

Front 357

Back 357

Cicatricial / Mucous Membrane Pemphigus

Front 358

Back 358

erythema multiforme

Front 359

Back 359

erythema multiforme

Front 360

Back 360

erythema multiforme

Front 361

Back 361

erythema multiforme

Front 362

Back 362

erythema multiforme

Front 363

Back 363

erythema multiforme

Front 364

Back 364

erythema migrans

Front 365

Back 365

erythema migrans

Front 366

Back 366

erythema migrans

Front 367

Back 367

reiters/reactive arthritis

Front 368

Back 368

reiters/reactive arthritis

Front 369

Back 369

lichen planus

Front 370

Back 370

lichen planus

Front 371

Back 371

drug induced lichen planus

Front 372

Back 372

drug induced lichen planus

Front 373

Back 373

erosive lichen planus

Front 374

Back 374

erosive lichen planus

Front 375

Back 375

reticular lichen planus

Front 376

Back 376

reticular lichen planus

Front 377

It is characterized by increased proliferation of cutaneous keratinocytes; etiopathogenesis poorly understood; probably caused by abnormal production of cytokines; genetic factors may have some role but may/must be influenced by environmental agents.

Back 377

psoriasis

Front 378

-years with periods of exacerbation and quiescence
- lesions worse during winter; scalp, elbows and knees are the favorite sites; lesions appear as well-demarcated erythematous plaques with a silvery scale; -usually asymptomatic these lesions may itch; approximately 4% of the patients with psoriasis develop arthritis

Back 378

psoriasis

Front 379

are oral lesion for psoriasis common?
how do oral lesions present themselves?

Back 379

no.
white/red ulcers

Front 380

What types levels of severity for psoriasis are treated as follows:

-corticosteroids, coal tar derivatives, keratolytic agents, UV light
-requires no tx
-Psoralen and UV-A (PUVA), methotrexate or cyclosporine

Back 380

-MODERATE:corticosteroids, coal tar derivatives, keratolytic agents, UV light
-MILD:requires no tx
-SEVERE: Psoralen and UV-A (PUVA), methotrexate or cyclosporine

Front 381

serious multisystem disease with variety of skin and oral manifestations; related to increased B lymphocyte activity and abnormal T cell function; cause is unknown but genetic factors may play a role

Back 381

SLE

Front 382

clinical features of ____ :

fever, wt. loss, arthritis & malaise; 40-50% have butterfly rash which gets worse with sunlight; 40-50% have kidney problems; approximately 50% have Libman-Sacks endocarditis.

Back 382

SLE

Front 383

what is the gender predilection and average age occurence for SLE?

Back 383

females
31yo

Front 384

-5-25% have oral lesions.
-Palate, buccal mucosa & gingivae usually involved.
- Lesions may have lichenoid appearance but may vary from nonspecific to granulomatous to ulcerative.
-Ulceration, pain, erythema and hyperkeratosis may occur.

Back 384

SLE

Front 385

_______skin lesions of SLE begin as scaly erythematous patches, which are made worse by sunlight; atrophy, scarring and hypopigmentation or hyperpigmentation is typically seen
- known as DISCOID L.E.

Back 385

Chronic Cutaneous Lupus Erythematous

Front 386

Skin lesions are most prominent feature and while photosensitive, the patients usually do not show induration and scarring as those with CCLE do.
what form of SLE _______, where arthritis is usually present but not the renal, cardiac or neurologic problems that are common in SLE.

Back 386

Subacute Cutaneous Lupus Erythematous

Front 387

what type of immunological test is highly diagnostic for both SLE and CCLE and the different types?

Back 387

direct immunofluorescence on LESION skin

Front 388

Progressive Systemic Sclerosis Scleroderma
Hide-Bound Disease
are synonyms for ___?

Back 388

systemic sclerosis

Front 389

-Characterized by dense collagen deposits in tissues of body
-skin develops a diffuse, hard, usually smooth texture and surface; “mast-like” and “mouse” facies develop.
-Fibrosis of lungs, heart, kidneys and GI tract may lead to organ failure.

Back 389

Systemic sclerosis

Front 390

Microstomia with limitation of mouth opening is seen in approximately 70% of the patients; “purse-string” appearance may develop; gingival recession may occur and loss of tongue mobility may lead to dysphagia

Back 390

systemic sclerosis

Front 391

Diffuse widening of PDL is often seen.
-Resorption of posterior ramus, coronoid and condyle may occur (in around 20% of patients).
-Mild form of disease called localized scleroderma usually affects only a patch of skin; “coup de sabre” (scar-like lesion) may be observed.

Back 391

systemic sclerosis

Front 392

Acrosclerosis; Limited Scleroderma are synonyms for?

Back 392

Crest Syndrome

Front 393

what are the predilections for CREST syndrome?

Back 393

females
50-60s

Front 394

what are the symptoms that CREST stand for?

Back 394

C= calcinosis
R= Raynaud's phenom
E=Esophageal dysmotility
S= Sclerodactyly
T = Telangiectasisa

Front 395

what symptom of CREST syndrome features:
affects patients hands and feet, they appear white due to blanching as the result of severe vasospasm, may later appear bluish to red-blue in color, results from cold and usually accompanied by pain.

Back 395

Raynaud's phenom

Front 396

demonstration of anti-centromere antibodies

Back 396

CREST syndrome

Back 397

psoriasis

Front 398

Back 398

psoriasis

Front 399

Back 399

psoriasis

Front 400

Back 400

psoriasis

Front 401

Back 401

SLE

Front 402

Back 402

SLE

Front 403

Back 403

systemic sclerosis

Front 404

Back 404

systemic sclerosis

Front 405

Back 405

systemic sclerosis

Front 406

Back 406

CREST syndrome

Front 407

Back 407

CREST sydrome

Front 408

Back 408

calcinosis of CREST syndrome

Front 409

Back 409

sclerodactyly of CREST syndrome

Front 410

Back 410

telangiectasia of CREST syndrome

Front 411

A heterogeneous group of heritable disorders characterized by impairment of collagen maturation.
Mutation of COL1A1 (chromosome 17) or COL1A2 (7).

Back 411

Osteogenesis Imperfecta

Front 412

most common type of heritable bone disease

Back 412

Osteogenesis Imperfecta

Front 413

clinical features:
osteopenia, bone fragility, (fractures), blue sclera, altered teeth, hypoacusis (hearing loss) long bone and spine deformity and joint hyperextensibility.

Back 413

Osteogenesis Imperfecta

Front 414

Oral features are:
dentinogenesis imperfecta, maxillary hypoplasia with class III malocclusion and radiographic lesions similar to florid cemento-osseous dysplasia.

Back 414

Osteogenesis Imperfecta

Front 415

what type of Osteogenesis Imperfecta is :
: AD; Most common and mildest form, 10% congenital fracture; 10% never fracture; blue sclera; hearing loss before 30; hyperextensibility and tooth defects variable.

Back 415

Type I

Front 416

What type of Osteogenesis Imperfecta is:
AR/AD; Most severe form; 90% stillborn or die before 4 weeks; blue sclera; opalescent teeth may be present.

Back 416

Type II

Front 417

What type of Osteogenesis Imperfecta is:
AD/AR: Moderate to severe bone fragility; blue sclera fades over time; hyperextensibility and hearing loss common; 2/3 die in childhood from cardiopulmonary failure secondary to kyphoscoliosis; some have opalescent dentin.

Back 417

Type III

Front 418

AD; Mild to moderate bone fragility-50% congenital fractures with frequency decreasing with time; blue sclera fades over time; opalescent dentin variable

Back 418

Type IV

Front 419

Albers-Schönberg Disease

Back 419

Osteopetrosis

Front 420

A group of rare hereditary skeletal defects of increased bone density resulting from defective remodeling due to failure in osteoclast function

Back 420

Osteopetrosis

Front 421

Endochondral, endosteal and periosteal growth without concomitant resorption results in thickening of bone.

Back 421

Osteopetrosis

Front 422

What form of Osteopetrosis is:
-AR; marrow failure, fracture and cranial nerve compression (blindness, deafness, facial paralysis.
Hepatosplenomegaly, infections and osteomyelitis.
Delayed tooth eruption; extraction osteomyelitis

Back 422

Infantile/malignant form

Front 423

What form of Osteopetrosis is:
-AD; limited mostly to axial skeleton; bone pain is common but nearly 40% are asymptomatic; marrow failure rare.
Variants with cranial nerve compression-without fracture or fracture without cranial nerve compression

Back 423

Adult/benign form

Front 424

What form of Osteopetrosis is malignant in form?

Back 424

Infantile

Front 425

Defect in CBFA1 gene (6p21); AD with 40% sporadic cases; defect in osteoblastic differentiation and bone formation.
Clavicles show hypoplasia or malformation; about 10% one/both clavicles are absent.
Short stature, large head with frontal & parietal bossing, hypertelorism, broad nose, depressed nasal bridge.
Persistent open fontanels and cranial sutures.

Back 425

Cleidocranial Dysplasia

Front 426

Dental & gnathic manifestations include narrow, high-arched palate w/o cleft, prolonged retention of deciduous teeth, delayed eruption of permanent teeth, numerous unerupted permanent and supernumerary teeth (lack secondary cementum). Narrow ramus, pointed coronoid processes, thin zygomatic arch, small/absent maxillary sinuses, acute gonial angle, prognathism.

Back 426

Cleidocranial Dysplasia

Front 427

Theories as to cause include aberrant bone regeneration following tooth extraction, persistent fetal marrow or marrow hypoplasia due to increased demand for erythrocytes.
Asymptomatic ill-defined circumscribed lucency from a few to several cm in size.

Back 427

Focal Osteoporotic Marrow Defect

Front 428

for Focal Osteoporotic Marrow Defect the site predilection is where in the oral cavity?

Back 428

posterior edentulous mandible

Front 429

what is the gender predilection for Focal Osteoporotic Marrow Defect ?

Back 429

female adults

Front 430

Focal area of increased radiodensity of unknown cause.
Also known as dense bone island, bone eburnation, bone whorl, bone scar, enostosis, focal (periapical) osteopetrosis.
-Well defined rounded or elliptic radiodense mass 3-20 mm.

Back 430

idiopathic osteosclerosis

Front 431

Back 431

osteogenesis imperfecta

Front 432

Back 432

osteogenesis imperfecta

Front 433

Back 433

osteogenesis imperfecta

Front 434

Back 434

osteogenesis imperfecta

Front 435

Back 435

Osteopetrosis

Front 436

Back 436

osteopetrosis

Front 437

Back 437

osteopetrosis

Front 438

Back 438

osteopetrosis

Front 439

Back 439

osteopetrosis

Front 440

Back 440

cleidocranial dysplasia

Front 441

Back 441

cleidocranial dysplasia

Front 442

Back 442

cleidocranial dysplasia

Front 443

Back 443

cleidocranial dysplasia

Front 444

Back 444

focal osteoporotic marrow defect

Front 445

Back 445

idiopathic osteosclerosis

Front 446

Back 446

idiopathic osteosclerosis

Front 447

Also known as Gorham disease, Gorham-Stout syndrome, vanishing bone disease and phantom bone disease.

Back 447

Massive Osteolysis

Front 448

Rare, idiopathic spontaneous progressive destruction of one or more bones; may represent hamangiomatosis of bone.

Pelvis, humeral head or shaft & axial skeleton most common sites.

Back 448

Massive Osteolysis

Front 449

what is the general age predilection for Massive Osteolysis?

Back 449

Most often in children & young adults; 50% report antecedent trauma.

Front 450

the following is characteristic of what condition?
Signs include mobile teeth, pain, malocclusion, midline deviation, pathologic fracture and obstructive sleep apnea.
Intramedullary lucency, loss of lamina dura, thinning of cortex.
Spontaneous arrest; radiation/surgery

Back 450

Massive Osteolysis

Front 451

What arch is most commonly affected by Massive Osteolysis

Back 451

Mandible

Front 452

Also known as osteitis deformans

Back 452

Paget's Disease

Front 453

Idiopathic disorder of abnormal anarchic resorption and deposition of bone (abnormal remodeling).

Back 453

Paget's

Front 454

what is the gender predilection for Paget's disease of bone?

Back 454

males

Front 455

what is the most common (form/presentation) of Paget's disease of bone?

Back 455

polyostic > monostotic

Front 456

if involved, which arch is most commonly affected by Paget's disease?

Back 456

maxilla

Front 457

Bowing of legs, leontiasis ossea, fracture, bone and joint pain, pressure neuropathy.
May cause progressive alveolar ridge expansion, spacing of teeth and “dental appliances that no longer fit the mouth”.
Altered trabecular pattern initially from decreased bone density evolves to patchy sclerotic areas having a “cotton wool” appearance

Back 457

Paget's

Front 458

Elevation of serum alkaline phosphatase treated if elevated by more than 25-50%; elevated urinary hydroxyproline levels.
Increased risk of osteosarcoma (1%) and giant cell tumors of bone.
Treatment with calcitonin & biophosphonate; plicamycin for refractory cases no longer used

Back 458

Paget's

Front 459

Radiographic lesions range from less than 5 mm to greater than 10 cm; unilocular or multilocular; differential diagnosis required.
Histopathologically consist of multinucleated giant cells in a stroma of spindle-shaped mesenchymal cells usually with numerous small vessel and hemorrhage—indistinguishable from hyperparathyroidism, cherubism and aneurysmal bone cyst.

Back 459

Central Giant Cell Granuloma

Front 460

What is the age and gender predilection for Central Giant Cell Granuloma?

Back 460

females
<30 yo

Front 461

What arch is one most likely to find a Central Giant Cell Granuloma lesion?

Back 461

mandible (anterior 2/3rds)

Front 462

is the Central Giant Cell Granuloma lesion neoplastic in nature?

Back 462

nope

Front 463

Which of the following is considered a true neoplasm and has a high recurrence rate and rate for metastases of 10%:

Central Gian Cell Granuloma
Giant Cell Tumor

Back 463

Giant Cell Tumor

Front 464

where are giant cell tumors often found?

Back 464

long bones

Front 465

Rare development condition of the jaws, generally AD (4p16) with high penetrance but variable expressivity.

Involved bone produces widening and distortion of alveolar ridges, tooth displacement, altered eruption, impaired mastication & speech difficulties.

Back 465

Cherubism

Front 466

Microscopically similar to CGCG; eosinophilic cuff-like deposits around small vessels a helpful finding. Long-standing lesions become more fibrous with fewer giant cells.

Back 466

Cherubism

Front 467

Also known as traumatic bone cyst, hemorrhagic bone cyst, solitary bone cyst & idiopathic bone cavity.

Back 467

Simple Bone Cyst

Front 468

A benign, empty or fluid-filled cavity within bone devoid of epithelial lining.

Back 468

Simple Bone Cyst

Front 469

Usually asymptomatic; 20% have swelling of jaw.
Well-delineated radiolucency; usually in premolar-molar region, often scalloped upward between the roots of teeth; rarely multilocular.
Pulp test teeth; can be associated with cemento-osseous dysplasia.

Back 469

Simple Bone Cyst

Front 470

Majority of ____ occur in long bones, ages 10-20; mandible; 60% of jaw lesions in males

Back 470

Simple Bone Cysts

Front 471

Back 471

massive osteolysis

Front 472

Back 472

massive osteolysis

Front 473

Back 473

paget's disease

Front 474

Back 474

paget's disease

Front 475

Back 475

paget's disease

Front 476

Back 476

paget's disease

Front 477

Back 477

paget's disease

Front 478

Back 478

paget's disease

Front 479

Back 479

paget's disease

Front 480

Back 480

paget's disease

Front 481

Back 481

paget's disease

Front 482

Back 482

central giant cell granuloma

Front 483

Back 483

central giant cell granuloma

Front 484

Back 484

central giant cell granuloma

Front 485

Back 485

central giant cell granuloma

Front 486

Back 486

central giant cell granuloma

Front 487

Back 487

giant cell tumor

Front 488

Back 488

cherubism

Front 489

Back 489

cherubism

Front 490

Back 490

cherubism

Front 491

Back 491

cherubism

Front 492

Back 492

cherubism

Front 493

Back 493

cherubism

Front 494

Back 494

simple bone cyst

Front 495

Back 495

simple bone cyst

Front 496

An intraosseous accumulation of variable-sized blood-filled spaces surrounded by cellular fibrous CT & reactive bone.

Back 496

Aneurysmal Bone Cyst

Front 497

are Aneurysmal Bone Cysts true neoplasms?

Back 497

nope

Front 498

what is the most common site for Aneurysmal Bone Cysts?

Back 498

shaft of long bone

Front 499

Possibly due to vascular disruption of bone:
-described aas ballooning out "blow - out" distension of bone contour

Back 499

Aneurysmal Bone Cyst

Front 500

20% of jaw lesions associated with other pathosis (fibro-osseous lesion of CGCG

Back 500

Aneurysmal Bone Cyst

Front 501

Developmental condition of replacement of normal bone by an excessive proliferation of cellular fibrous connective tissue intermixed with irregular bone trabeculae.

Back 501

Fibrous Dysplasia

Front 502

-Sporadic condition attributed to mutation of GNAS 1 gene
-clinical severity determined by time when the mutation occurs; early embryonic mutation produces polyostotic disease with other abnormalities; postnatal monostotic disease.

Back 502

Fibrous Dysplasia

Front 503

what is the most common form of Fibrous Dysplasia and which arch is most affected?

Back 503

monostotic
maxilla

Front 504

what form of Fibrous Dysplasia presents with:
-Radiographic poorly demarcated “ground-glass” appearance of affected bone causing expansion; in long bones often radiolucent, multilocular & expansile.
PDL may be narrow and lamina dura indistinct; inferior alveolar nerve canal displaced superiorly.

Back 504

monostotic

Front 505

what type of Fibrous Dysplasia can be associated with Jaffe-Lichtenstein syndrome or McCune-Albright syndrome and associated cafe aulait spots?

Back 505

Polystotic (PFD)

Front 506

what is a clinical concern for fibrous dysplasia patients?

Back 506

high recurrence rate (25-50%)
osteosarcoma development

Front 507

Cellular connective tissue containing a mixture of woven bone, lamellar bone and cementum-like particles; ratio of fibrous tissue decreases over time as it becomes sclerotic by fusion of calcified elements

Back 507

Cemento-Ossesous Dysplasias

Front 508

what is the gender predilection for Focal Cemento-Osseous Dysplasias?

Back 508

females 9:1

Front 509

Solitary
radiolucent to radiopaque lesion, less than 1.5 cm, if opaque with thin radiolucent rim;
most often in posterior mandible; well-defined with slight irregular borders.

Back 509

Focal Cemento-Osseous Dysplasias

Front 510

F:M, 14:1.
Asymptomatic, anterior mandible, typically multifocal, periapical to vital teeth. Must pulp test teeth.
Lucent > opaque over time; < 1cm; PDL intact, opacity not fused to tooth.
No treatment necessary

Back 510

Periapical Cemento-Osseous Dysplasias

Front 511

Multifocal involvement but not limited to the anterior mandible; often bilaterally symmetric.
Most often asymptomatic; pain, swelling or signs of osteomyelitis.

Back 511

Florid Cemento Osseous Dysplasia

Front 512

Florid Cemento Osseous Dysplasia gender predilection?

Back 512

females

Front 513

Uncommon hereditary AD disorder of gnathic bone in which massive sclerotic masses of disorganized mineralized tissue develop.
Begin as multiple periapical lucencies which expand to produce a mixed radiolucent-radiopaque pattern.
Anemia and multiple polypoid adenomas of the uterus reported in some affected females.

Back 513

Familial Gigantiform Cementoma

Front 514

Back 514

aneurysmal bone cyst

Front 515

Back 515

aneurysmal bone cyst

Front 516

Back 516

fibrous dysplasia

Front 517

Back 517

fibrous dysplasia

Front 518

Back 518

fibrous dysplasia

Front 519

Back 519

fibrous dysplasia

Front 520

Back 520

focal cemento osseous dysplasia

Front 521

Back 521

focal cemento osseous dysplasia

Front 522

Back 522

florid cemento osseous dysplasia

Front 523

Back 523

periapical cemento osseous dysplasia

Front 524

Back 524

periapical cemento osseous dysplasia

Front 525

A true neoplasm of an admixture of fibrous tissue & bony trabeculae, cementum-like spherules, or both

Small lesions asymptomatic; larger lesions produce painless swelling.
Well defined radiolucency, often with a sclerotic border, containing varying amounts of opacity. Larger lesions produce downward bow of the inferior cortex of mandible.

Back 525

Ossifying Fibroma

Front 526

What is a another name of Ossifying Fibroma?

Back 526

Cemento-Ossifying Fibroma

Front 527

Ossifying Fibroma is most commonly seen in 20s and 30s and has a ____ gender predilection

Back 527

female

Front 528

what is the site predilection for Ossifying Fibroma?

Back 528

PM-Molar region of mandible

Front 529

Richly cellular fibrous tissue showing strand of osteoid or psammomatoid ossicles.
Growth of individual tumors varies from slow to rapid; tends to be rapid in younger patients as compared to analogous condition in adults

Back 529

Juvenile Ossifying Fibroma

Front 530

Benign tumors (neoplasm?) of mature compact or cancellous bone that are restricted to the craniofacial skeleton.

Back 530

Osteoma

Front 531

-May be periosteal or endotseal; periotseal lesions produce slowing growing mass; endosteal lesions asymptomatic or slow enlargement.
-Condylar lesion produces lobulated mass; deviation of midline.
-Radiographically a circumscribed sclerotic mass

Back 531

Osteoma

Front 532

What is the syndrome that may include osteomas as a finding?
-Rare inherited AD disorder (gene on chromosome 5)
Within the spectrum of familiar colorectal polyposis
1 in 8,300-16,000
Osteomas of any bone, skull, paranasal sinuses and mandible common.
Supernumerary teeth in 20%

Back 532

Gardner Syndrome

Front 533

A syndrome that necessitates the following tx:
Colonectomy; elective prophylactic thyroidectomy, elective removal of osteomas; cysts and other tumors.

Back 533

Gardner Syndrome

Front 534

-Rare neoplasms of bone (<1%) are microscopically identical;
-most frequent in vertebral column, sacrum, calvarium, long bones small bones of hands/feet
--Painful radiolucent/opaque lesion often 2-4 cm

Back 534

osteoblastoma

Front 535

-Rare neoplasms of bone (<1%)
-most often femur, tibia & phalanges
-Painful radiolucent/opaque lesion often 2-4 cm

Back 535

Osteoid Osteoma

Front 536

which of the following neoplasms produces prostaglandins and thus can be relieved by aspirin?

Back 536

osteoid osteoma

Front 537

Osteoblastomas and osteoid osteomas have a site predilection for the ______ arch and are typically found in those younger than 30 yo

Back 537

mandibular (post.)

Front 538

Rare neoplasm thought by some to be identical with osteoblastoma differing only by its attachment to the root of a tooth. <1% of odontogenic tumor.

Back 538

Cementoblastoma

Front 539

the arch predilection of Cementoblastomas is the ____

Back 539

mandible (75%)

Front 540

-Pain reported by 2/3 of time
-Opaque mass fused to root surrounded by thin radiolucent rim.

Back 540

CementoblastomaRadiolucent with central area of opacity

Front 541

Benign neoplasm of hyaline cartilage

Back 541

Chondroma

Front 542

-Radiolucent with central area of opacity
-multiple lesions associated with Ollier disease and Maffuci syndrome

Back 542

Chondroma

Front 543

Chondromas are commonly found in:

condyle
jaw
or anterior maxilla?

Back 543

condyle and anterior maxilla

Front 544

Uncommon benign neoplasm (<1%) most often involving metaphysis of long bones; jaw lesions are rare and can be confused with chondromyxoid fibroma

Back 544

chondromyxoid fibroma

Front 545

Rare benign, non-neoplastic arthropathy of unknown cause; cartilaginous nodules form within the synovial membrane.

Back 545

synovial chondromatosis

Front 546

Uncommon bone tumor of fibroblastic tissue
Metaphyseal region of humerus or tibia most common; for jaw lesions, 90% in molar-ramus region of mandible.
Most case before age 30; for jaws the mean age is 14.
Painless swelling with radiolucency of bone.
Composed of bland collagenous connective tissue.
Sometimes locally destructive and aggressive; managed surgically according to aggressiveness

Back 546

Desmoplastic Fibroma

Front 547

what is the most common primary malignancy of bone?

Back 547

Osteosarcoma

Front 548

Most osteosarcomas are:
-periosteal
-intramedullary
-peripheral (juxtacortical)
-parosteal.

Back 548

intramedullary

Front 549

Most often between age _____ and located in distal femur or proximal tibia; secondary group > age 50 associated with _______located in axial skeleton

Back 549

10-20 yo
Paget's disease

Front 550

Back 550

ossifying fibroma

Front 551

Back 551

osteoblastoma or osteoid osteoma

Front 552

Back 552

chondromyxoid fibroma

Front 553

Back 553

synovial chondromatosis

Front 554

osteosarcomas are most commonly found in what arch?

Back 554

maxilla = mandible

Front 555

Osteoblastic, chondroblastic and fibroblastic are the most common microscopic subtypes of what disease?

Back 555

osteosarcomas

Front 556

What is the very common finding of those with osteosarcoma apart from:
-radiographic lesions of jaws vary from lucent to opaque
-often ill-defined
-spiking or roots
-osteophytic reaction (sun-ray) is seen in 25%.

Back 556

widening of PDL

Front 557

osteosarcoma metastases are found in:
-regional lymph nodes
-brain
-lung
-long bones
-and less commonly ______

Back 557

jaw

Front 558

what has the best prognosis of osteosarcoma types:

-juxtacortical or intramedullary?

Back 558

juxtacortical

Front 559

there is a 0.2% risk of post-radiation sarcoma for dose osteosarcoma pts. at a level of ______ cGy

Back 559

7000

Front 560

Malignant neoplasm of bone (10%) characterized by the formation of cartilage.

Back 560

chondrosarcoma

Front 561

are jaw lesions common for chondrosarcomas?

when they do occur what is the arch predilection?

Back 561

no (1-3%)

maxilla

Front 562

Extragnathic lesions of chondrosarcoma found in ileum, femur & humerus are often found later than what age?

Back 562

50 yo

Front 563

Sarcoma that most often produces a painless mass; may be associated with loosening of teeth, epistaxis, nasal obstruction and visual disturbance.
Lesions grades from I-IV; influence on outcome; most jaw lesion grade I or II

Back 563

chondrosarcoma

Front 564

Primary malignancy of bone of uncertain histogenesis with recent evidence of neuroendocrine origin.
85-90% show reciprocal translocation [t(11;22) (q24;q12)].
6-8% of primary malignancies of bone; long bones, pelvis and ribs; jaw or craniofacial lesions are very uncommon (1-2%).

Back 564

Ewings Sarcoma

Front 565

Rare in blacks.
Pain, paresthesia and swelling with loosening of teeth.
Ill-defined lytic lesion; “onion-skin” periosteal reaction of long bones rare in jaws.
Small round cells; PAS positive diastase labile; angiotropism and necrosis; MIC2 glycoprotein detected by immunoperoxidase (CD99).
Combined surgery, multidrug chemo & radiotherapy 5-year survival 40-80; pelvis worst.

Back 565

Ewings Sarcoma

Front 566

What is the age and gender predilection of Ewings Sarcoma?

Back 566

<20yo
males

Front 567

80% of reported metastatic tumors of jaw cases are found in the ____
Symptoms include pain, loosening of tooth, a mass or paresthesia (numb-chin syndrome).
Lucent lesion, well or ill defined (“moth-eaten”)

Back 567

mandible

Front 568

In general, metastatic carcinomas (especially breast & prostate) may stimulate new bone formation with a resultant radiopaque or mixed lesion with the most common sites being:
-breast
-prostate
3) ??
4) ??
5) ??

Back 568

lung
thyroid
kidney

Front 569

50% of the time metastases of jaw (or in general) can serve as the first sign of _______

Back 569

systemic disease

Front 570

why are metastases to the jaw found most often on the tooth bearing areas, particularly the molar regions of the mandible?

Back 570

they have a greater blood supply

Front 571

Metastases of Jaw for :
Adults: usually from breast, lung, colon, prostate, kidney, thyroid, testis

whereas:
Children: usually from adrenal neuroblastoma, _______ and ______

Back 571

embryonal rhabdomyosarcoma, Wilms’ tumor

Front 572

Back 572

osteosarcoma

Front 573

Back 573

osteosarcoma

Front 574

Back 574

osteosarcoma

Front 575

Back 575

osteosarcoma

Front 576

Back 576

osteosarcoma

Front 577

Back 577

osteosarcoma

Front 578

Back 578

osteosarcoma