6 Oral Manifestations of Systemic Diseases Flashcards
1
Q
dense collagen is deposited in the body in high amounts (immune mediated)
A
scleroderma
2
Q
- women 3x more than men
* adults
A
scleroderma
3
Q
- skin develops diffuse hard texture with a smooth surface
* localized, limited, or diffuse forms
A
scleroderma
4
Q
Facial skin:
• increased deposition of subcutaneous collagen
• smooth, taut, masklike
A
scleroderma
5
Q
fibrosis of lungs, heart, kidneys, GI tract —> leading to organ failure
A
scleroderma
6
Q
In patients with scleroderma, this is the result of collagen deposition in the perioral tissues. 70% of patients have limited opening.
A
microstomia
7
Q
- claw-like appearance of hands
- resorption of terminal phalanges
- ulceration of the finger tips due to collagen deposition and lack of blood supply
A
scleroderma
8
Q
vasoconstriction triggered by cold or emotional stress
A
Raynaud’s phenomenon (scleroderma)
9
Q
Initially: blanching of the digits
Minutes later: bluish due to venous stasis
Warming results in increased blood flow and change to a red hue (can be painful)
A
Raynaud’s phenomenon (scleroderma)
10
Q
Radiograph:
• widening of the PDL
• resorption of the posterior ramus, coronoid process, chin, and condyle
A
scleroderma
11
Q
survival rate of scleroderma (2, 8, and 12 yr survival)
A
80% 2 year
30-50% 8 year
12-30% 12 year
12
Q
tx for scleroderma
A
no tx available
13
Q
clinical signs of jaundice
A
- excess bilirubin in the bloodstream and accumulates in the tissues
- yellow discoloration of the skin and mucosa
*these are NOT specific signs, require physical examination and laboratory studies to determine the precise cause
14
Q
What is bilirubin derived from?
A
the breakdown of hemoglobin
15
Q
process of making bilirubin
A
- erythrocyte lifespan = 120 days
- physiologic breakdown of hemoglobin
- hemoglobin is degraded and processed by reticuloendothelial system
- unconjugated bilirubin released into bloodstream
16
Q
unconjugated vs conjugated bilirubin
A
Unconjugated—taken up by hepatocytes, conjugated with glucuronic acid
Conjugated—soluble product that can be excreted in the bile
17
Q
physiologic vs pathological causes of jaundice
A
Physiologic
• Jaundice at birth—low level of activity of the enzyme system that conjugates bilirubin
Pathologic
• Increased production of bilirubin—RBCs broken down too fast, liver cannot keep up processing (sickle cell anemia)
• Liver function abnormality—decreased uptake of the bilirubin from circulation, decreased conjugation in the liver cells
18
Q
2 main pathological causes of jaundice:
A
1) Liver function disturbance—forces conjugated bilirubin into the bloodstream (infection—viral hepatitis, alcoholic hepatitis, cancer)
2) Occlusion of the bile duct—gallstones, stricture, cancer
19
Q
Tissues that have a high content of elastin and therefore attract bilirubin:
A
1) sclera—the first site at which the yellow color is noted
2) lingual frenum
3) soft palate
20
Q
group of conditions characterized by deposition of amyloid
A
amyloidosis
21
Q
What is amyloid?
A
extracellular proteinaceous substance
22
Q
Amyloid can be formed in multiple ways (2)
A
- from immunoglobulin light chains = AL
* from beta2-microglobulin = Abeta2M (protein that cannot be removed by dialysis, accumulates in plasma)
23
Q
protein that cannot be removed by dialysis, accumulates in plasma
A
beta2-microglobulin (due to hemodialysis-associated amyloidosis)
24
Q
All amyloid proteins form into this molecular configuration and then are deposited into tissues.
A
beta-pleated sheet
25
Two main categories of clinical presentation of amyloidosis:
1) Organ-limited—a solitary nodule
| 2) Systemic—primary, myeloma associated, secondary, hemodialysis associated, heredofamilial
26
Name the systemic amyloidosis: most common type, idiopathic
primary—AL
27
Name the systemic amyloidosis: excess light-chain proteins from myeloma tumor cells accumulate and are deposited in tissues
myeloma-associated—AL
28
Name the systemic amyloidosis: 15-20% of all amyloidosis cases
myeloma-associated—AL
29
Name the systemic amyloidosis: beta-2 microglobulin is a normally occurring protein that is not removed by the dialysis process —> accumulates in the plasma —> forms deposits in bones and joints (carpal tunnel, cervical spine)
*improving with better dialyzers in the future
hemodialysis-associated amyloidosis
30
Name the systemic amyloidosis: primarily autosomal dominant
heredofamilial
31
Clinical manifestations:
• average age = 65
• initial symptoms are nonspecific (fatigue, weight loss, hoarseness, orthostatic hypotension)
• eventually—carpal tunnel syndrome, hepatomegaly, mucocutaneous lesions, macroglossia (10-40% of patients)
primary and myeloma-associated amyloidosis
32
deposition of amyloid in the tongue, may see ulceration
macroglossia due to primary and myeloma-associated amyloidosis
33
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Skin lesions:
• smooth-surfaced, firm, waxy papules and plaques
• eyelid
• retroauricular
• neck
• lips
• petechia
```
Also:
• heart deposits
• kidney deposits
amyloidosis
34
biopsy of amyloidosis
* rectal biopsy 80%+
* abdominal fat aspiration 55-75%
* labial salivary gland 80%+
Biopsy reveals amorphous acellular eosinophilic material
• stain with congo red
• polarized light = apple green color
35
tx of amyloidosis
depends on cause of the amyloid, many cases no tx available
36
prognosis is guarded to poor, most patients die of cardiac failure, arrhythmia, or renal disease within months to a few years after the dx
amyloidosis
37
possible causes of iron deficiency anemia (4)
* excessive blood loss
* increased demand for RBCs (growth, pregnancy)
* decreased intake of iron
* decreased iron absorption (celiac sprue)
38
Iron deficient anemia:
% of women of childbearing age
% of men (What is the main cause?)
11% of women
2% of men, GI disease is main cause (peptic ulcer, diverticulosis, hiatal hernia, malignancy)
39
* fatigue, palpitations, lightheadedness
* angular cheilitis
* diffuse or patchy atrophy of dorsal tongue papillae
* possible tenderness or burning sensation of oral mucosa
iron deficient anemia
*confirm dx with CBC
40
iron-deficiency anemia in conjunction with glossitis and dysphagia
Plummer-Vinson syndrome
41
Plummer-Vinson syndrome is associated with a high frequency of?
oral and esophageal SCC
*considered a premalignant process
42
most patients are women of Scandinavian or Northern European background
Plummer-Vinson syndrome
43
Systemic findings: fatigue, shortness of breath, weakness
Oral findings: burning sensation of tongue and oral mucosa, pain can be so severe that dentures cannot be worn, angular cheilitis may be severe, atrophy of the lingual papillae, smooth red appearance of the dorsal tongue
Plummer-Vinson syndrome
44
esophageal webs—frequent difficulty swallowing (dysphasia) or pain on swallowing, endoscopy or esophageal barium reveals abnormal bands of tissue in the esophagus
Plummer-Vinson syndrome
45
altered growth of nails (spoon-shaped, brittle) seen with Plummer-Vinson syndrome
koilonychia
46
tx of Plummer-Vinson syndrome
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Dietary iron supplementation
• resolves the anemia
• relieves the glossodynia
• may reduce esophageal symptom severity
• esophageal dilation if needed
```
47
Why is a periodic evaluation for oral, pharyngeal, and esophageal cancer necessary for Plummer-Vinson syndrome?
5-50% prevalence of upper aerodigestive tract malignancy has been reported in affected persons
48
cause of pernicious anemia
poor absorption of extrinsic factor/vitamin B12/cobalamin
49
allows for absorption of B12, produced by parietal cells of the stomach
intrinsic factor
50
Why are mitotically active cells the most dramatically affected by pernicious anemia?
B12 is required for normal nucleic acid synthesis
51
Systemic symptoms:
• fatigue, weakness, SOB, headache
• tingling, numbness of extremities
pernicious anemia
52
* burning sensation of tongue, lips, buccal mucosa
* patchy areas of erythema and atrophy
* 50-60% of patients have tongue involvement
pernicious anemia
53
How to test for pernicious anemia?
* bloodwork shows macrocytic anemia and reduced serum cobalamin levels
* serum antibodies directed against intrinsic factor—quite specific for pernicious anemia
* Schilling test—compares absorption and excretion rates of radiolabeled cobalamin (complicated to perform, now considered obsolete)
54
test for pernicious anemia that compares absorption and excretion rates of radiolabeled cobalamin (complicated to perform, now considered obsolete)
Schilling test
55
tx for pernicious anemia
* monthly IM injections of cyanocobalamin —> rapid response, reports of clearing oral lesions ~ 5 days
* high-dose oral cobalamin therapy (equally effective)
56
advantages of high dose oral cobalamin vs monthly IM injections of cyanocobalamin for treating pernicious anemia
cost-effectiveness, elimination of injections
57
gastric carcinoma 7x more likely in patients with this
pernicious anemia
58
increased risk of malignancy for people with pernicious anemia
gastric carcinoma 7x more likely
59
3 parathyroid disorders:
hypoparathyroidism
primary hyperparathyroidism
secondary hyperparathyroidism
60
results from decreased secretion of PTH
hypoparathyroidism
61
most common cause of hypoparathyroidism
removal of the parathyroids during thyroidectomy
62
symptoms of hypoparathyroidism (3)
1) hypocalcemia—causes increased neuromuscular excitability (mild tingling of hands and feet, parenthesia, muscle cramps/spasms)
2) dental related changes—if during tooth formation then pitting enamel hypoplasia, failure of tooth eruption)
3) mental effects—hyperirritability, fatigue, anxiety, mood swings/personality disturbances
63
tx for hypoparathyroidism
oral calcium and vitamin D precursor
64
syndrome caused by excessive secretion of PTH resulting from intrinsic parathyroid disease
primary hyperparathyroidism
65
2 causes of primary hyperparathyroidism:
1) adenoma of the parathyroid gland (80% of cases)
| 2) hyperplasia parathyroid glands (15-20% of cases)
66
Name the cause of primary hyperparathyroidism: dramatic enlargement of 1 of the 4 glands
adenoma of parathyroid gland
67
tx of adenoma of parathyroid gland
surgical removal of the tumor
68
Name the cause of primary hyperparathyroidism: develops in patients with familial multiple endocrine neoplasia types I and II, all 4 glands are involved
hyperplasia parathyroid glands
69
> 60 yo, females 2-4x more often
primary hyperparathyroidism
70
hypercalcemia on blood analysis
primary hyperparathyroidism
71
Why does primary hyperparathyroidism show hypercalcemia on blood analysis?
increased PTH production —> excess loss of calcium from the bones —> increased serum calcium, decreased serum P
72
bones, stones, moans, groans
primary hyperparathyroidism
73
painful bones—primary hyperparathyroidism
* bone resorption (finger tips) —> subperiostel resorption
* generalized loss of the lamina dura
* ground glass appearance of bone
74
renal stones—primary hyperparathyroidism
* renal tubules absorb more calcium
* PTH stimulates the tubules to produce active vitamin D
* active vitamin D promotes more calcium absorption from the intestines
75
abdominal groans—primary hyperparathyroidism
duodenal ulcers, gallstones
76
moans—primary hyperparathyroidism
depression and lethargy
77
intrabony tumor called a “Brown tumor”, named because grossly tissue is deep red to brown color
primary hyperparathyroidism
78
well-demarcated, multilocular radiolucency
Brown tumor (primary hyperparathyroidism)
79
locations of Brown tumor
mandible, clavicle, ribs, pelvis
80
histopathologically identical to giant cell granuloma, numerous multinucleated giant cells
Brown tumor (associated with primary hyperparathyroidism)
81
excess secretion of PTH as a response to chronically low calcium levels (hypocalcemia)
secondary hyperparathyroidism
82
causes of secondary hyperparathyroidism (5)
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Chronically low calcium levels due to:
• kidney failure requiring dialysis (most common)—GFR falls, leads to increased phosphate retention, in turn leads to increased PTH production
• stomach or intestine bypass surgery
• celiac disease
• Chron’s disease
• severe vitamin D deficiency
```
83
because there is not enough calcium the parathyroids go into overdrive and become hyperplastic, all 4 glands become enlarged
secondary hyperparathyroidism
84
excess PTH production results in?
calcium resorbed from bones
85
term specifically for bone resorption in patients with renal failure and secondary hyperparathyroidism
* bone and joint pain
* bone deformation and fractures at late stages
renal osteodystrophy
86
generalized loss of lamina dura, blurring of the trabecular pattern leads to “ground glass” appearance
secondary hyperparathyroidism
87
tx for secondary hyperparathyroidism
* optimize levels of serum calcium and phosphate (dietary restriction of phosphorous and phosphate binders)
* control PTH and vitamin D by use of vitamin D analogues
* surgical removal of the glands
* renal transplant if cause is renal failure
88
rare metabolic bone disease due to tissue-nonspecific alkaline phosphatase deficiency
hypophosphotasia
89
plays a role in the production of bone, precise mechanism is unknown, deficiency causes hypophosphotasia
alkaline phosphatase
90
one of the first presenting signs of hypophosphotasia and why
* premature loss of the primary teeth
| * likely caused by a lack of cementum on the root surfaces
91
____ distinct mutations of the gene responsible for alkaline phosphatase causing hypophosphotasia
150
92
2 genetic causes of hypophosphotasia:
Homozygous autosomal recessive form
• severe manifestations
• patients usually identified in infancy
```
Milder forms
• autosomal dominant or recessive
• variable degrees of expression
• appearing in childhood or adulthood
• younger the age of onset = more severe
```
93
How many types of hypophosphotasia are now recognized, depending on the severity and the age of onset of the symptoms?
6
94
3 common factors in all types of hypophosphotasia:
* reduced levels of alkaline phosphatase isozyme found in the bone, liver, and kidney
* increased levels of blood and urinary phosphoethanolamine
* bone abnormalities that resemble rickets
95
one of the first presenting signs of childhood hypophosphotasia? why? radiographic signs?
* premature loss of the primary teeth without evidence of significant inflammatory issue (incisors may be the first and only teeth involved)
* likely caused by lack of cementum on root surfaces
* teeth may show enlarged pulp chambers, may see alveolar bone loss
96
dx of hypophosphotasia
* based on the clinical manifestations
* decreased levels of serum alkaline phosphatase
* increased amounts of phosphoethanolamine in both the urine and blood
97
tx of hypophosphotasia
* alkaline phosphatase infusions are unsuccessful
* fractures require orthopedic surgery
* prostheses for missing teeth, implants can be used
