Oral Manifestations of Systemic Disease Flashcards
1
Q
What are Gastrointestinal Diseases
what are example of them?
A
Inflammatory bowel diseases associated with oral findings
▪ Crohn disease
▪ Ulcerative colitis
▪ Pyostomatitis vegetans
▪ Celiac Disease
2
Q
Crohn Disease
Regional Ileitis
What areas does affect?
What are the symptoms?
A
What areas does affect?
● Primarily affecting distal small intestine and proximal colon
What are the symptoms?
● Symptoms include abdominal cramping, pain, bloating, diarrhea, and nauseas (similar amongst the GI diseases)
● Patients often have weight loss and malnutrition
● 20% have abrupt onset of symptoms resembling acute appendicitis or bowel perforation
3
Q
Crohn Disease
Regional Ileitis
When it is diagnosed?
Etiology?
Prevalence?
What are the oral implactions?
A
When it is diagnosed?
● Usually initially diagnosed in adolescents
Etiology?
● Etiology unknown‐ immunologically mediated?
o Theory of being too clean as a child and having a negative response as we grow older since we’re not used to normal bacteria
Prevalence?
● Prevalence increasing, reason unknown
What are the oral implactions?
●Oral lesions can be first sign of disease
4
Q
Crohn Disease
Histopathology
A
● Superficial or deep ulceration with adjacent granulation tissue extending into deep submucosa or below
● A transmural granulomatous inflammation
o Sarcoid‐like, non‐caseating, poorly formed granulomas, in all tissue layers (50‐70% of cases) usually adjacent to blood
vessels or lymphatics
● Transmural inflammation with lymphoid aggregates throughout bowel wall
5
Q
Crohn Disease
Oral Findings
A
- Recurrent oral ulcerations can mimic those seen with recurrent aphthous lesions
-
Diffuse or nodular swelling of the oral and perioral tissues
- Can look like epulis fissuratum
-
Deep linear granulomatous‐appearing ulcerations (often in the buccal vestibule area)
- Cobblestone mucosal appearance
- Polypoid tag‐like lesions on vestibular and retromolar mucosa
- Enlargement of lips caused by granulomatous inflammation: orofacial granulomatosis
6
Q
Crohn Disease
Treatment
A
- Current strategies aim for deep and long‐lasting remission, with the goal of preventing complications, such as surgery, and blocking disease progression
- Immunosuppressant such as cyclosporine
- In more severe cases;
- *high dose corticosteroids and**
- chemotherapeutics to induce a remission
-
Nutritional supplements (iron, folate)
- Because they are unable to absorb nutrients
- If medical means do not keep patient under control► surgical removal of a portion or all of the intestine
- When intestinal symptoms are under control►oral ulcerations resolve
7
Q
Which systemic disease manifests like this?
A
Crohn Disease
- Patients can also get angular cheilitis
- Above the Linear ulceration, can see a flap like structure which is the hyperplastic margin
8
Q
Which systemic disease has this oral manifestation?
A
- we see the ulceration and hyperplastic tissue surrounding it.
9
Q
Which systemic disease has this oral manifestation?
A
Crohn Disease
Nodular appearance of buccal mucosa
10
Q
Which systemic disease has this oral manifestation?
A
Crohn Disease
we see more nodules
11
Q
Which systemic disease has this oral manifestation?
A
Crohn Disease
Linear granulomatous ulcerations
But they are not the aphthous ulcerations but the more
linear type
12
Q
Ulcerative Colitis
- What are the symptoms
- What type of Cancer risk it presents?
A
What are the symptoms
- A chronic inflammatory disease of the colon (mucosa and submucosa) presenting with diarrhea, rectal bleeding, abdominal pain, weight loss and fever
What type of Cancer risk it presents?
- Increased risk of colon cancer
13
Q
Ulcerative Colitis
Oral Lesions
A
● In some cases, patients get recurrent oral ulcerations (can have aphthous‐like lesions)
● Papillary mucosal projections with deep linear ulcers and fissures
● Intraepithelial pustules of the mucosa (pyostomatitis vegetans)
14
Q
Ulcerative Colitis
Management
A
● Use of anti‐inflammatory medications
o Sulfasalazine or Prednisone
● If medical means do not succeed► then removal of part or all of colon
15
Q
How different is Ulcerative Collitis from Crohn Disease?
A
Unlike Crohn’s, lesions extend in a continuous fashion proximally from the rectum (no skip lesions) and histologically don’t have granulomas
16
Q
What is the difference between Inflammatory Bowel
Disease IBD & Irritable
Bowel Syndrome IBS
A
● IBD
o Classified as a disease
o Can cause destructive inflammation and permanent harm to the intestines
o The disease can be seen during diagnostic imaging
o Increased risk for colon cancer
● IBS
o Classified as a syndrome, a group of symptoms
o Dose not cause inflammation; rarely requires hospitalization or surgery
o There is no sign of abnormality during an exam of the colon
▪ Usually because it’s only periodic
o No increased risk form colon cancer or IBD
17
Q
Pseudomembranous
Ulcerative Colitis
Causes?
A
Bactrial Overgrowth in the pseduomemberane
(C.difficile Overgrowth)
Causes:
- Clindamycin prolonged use ~2 weeks can cause the C. difficile overgrowth.
- Always warn patients if you prescribe clindamycin about possible side effects and stop usage since do not want them to develop untreatable strains
18
Q
Pyostomatitis
Vegetans
what is it?
Demographics?
Symptoms onset?
A
What is it?
- Thought to be an unusual presentation of inflammatory bowel disease, especially ulcerative colitis (sometimes with Crohn’s)\
Demographics
- In only a rare subset of patients
- Typically present before 30 years of age
Symptoms onset
- ~25% of cases seen in absence of GI symptoms
- May see oral symptoms before the GI symptoms
19
Q
Pyostomatitis
Vegetans
Oral Symptoms
and
Most common sites in the mouth?
A
Oral Symptoms
● Recurrent oral ulcerations _concurrent with, or prior to GI symptoms_
● Oral mucosa is erythematous and thickened with multiple
cream/yellow‐colored pustulesandsuperficial erosions
● Linear “snail track” oral pustules
Most common sites in the mouth?
● Most common sites include buccal and labial mucosa, soft palate, and ventral tongue
20
Q
Pyostomatitis
vegetans
Treatment
A
● Treatment is not well standardized, fairly rare disease and good double blind studies rare
● Can use topical corticosteroids
● Werchniak et al had good results with topical tacrolimus
● Sulfasalazine or Prednisone for GI lesions
● If GI symptoms are under control► oral lesions will resolve
21
Q
Oral Manifestations of which systemic disease?
A
Pyostomatitis
Vegetans
22
Q
Oral Manifestations of which systemic disease?
A
Pyostomatitis
vegetans
23
Q
Oral Manifestations of which systemic disease?
A
Pyostomatitis
vegetans
Snail track
appearance
24
Q
Celiac (Sprue) Disease
What is it?
Which gene is involved?
Symptoms?
A
What is it?
● Chronic disease (diffuse enteritis) of the small intestine which improves upon withdrawal of gluten proteins
Which gene is involved?
● >90% express HLA‐B8 histocompatibility antigen
Symptoms?
● Patients present with diarrhea, gas, weight loss, fatigue, impaired nutrient absorption, etc
25
Patients with **Celiac (Sprue) Disease** have risk of _developing which cancer?_
● 10‐15% risk of **GI lymphoma**
26
Celiac (Sprue) Disease
_Oral Symptoms_
● 10‐15% risk of GI lymphoma
● Oral symptoms include aphthous‐like ulceration of mucosa
● Patients can have enamel defects and pitting
27
**Amyloidosis**
What is it?
From what does it form?
Seen with what disease?
**What is it?**
* _Protein deposits in tissue_
* Paucicellular eosinophilic deposits amorphous eosinophilic deposit
**From what does it form?**
* Form **fibrillar β‐pleated sheets** within the tissue
Seen with what disease?
* **multiple myeloma**
28
**Amyloidosis**
what are its Types and what they are associated with?
1. **▪ _Primary amyloidosis_** is associated with **multiple myeloma**
2. **Reactive systemic amyloidosis**
3. **Hemodialysis** associated **amyloidosis kidney dysfunction**
4. **Hereditary amyloidosis** **(Familial Mediterranean Fever)**, present with **polyneuropathies, cardiac arrhythmias, renal failure, CHF**
5. **Localized dermal amyloidosis**
29
**Primary amyloidosis** is associated with **which cancer?**
**multiple myeloma**
30
Hemodialysis associated amyloidosis leads to ------
kidney dysfunction
31
**Amyloidosis**
**Organ Limited**
_Clinically_
* **Rare in oral cavity**
* Amyloid _nodule_, **asymptomatic** submucosal deposit
* **Not** associated with ***systemic symptoms***
32
**Primary or Muliple Myeloma associated Amyloidosis**
Systemic
_Demographics & Clinical presentations_
* Older adults **\> 65yrs**
* **Male** predilection
* Amyloid deposits _lead to_ **_macroglossia, carpal tunnel_ syndrome, hepatomegaly, dry mouth**
* **Skin lesions:** Waxy papules and plaques, **smooth surface (eyelid area, retroauricular, neck, lips), orangy, red appearance**
33
**Secondary Amyloidosis**
systemic
_Etiology & Effects_
* Due to _chronic inflammatory process_
* *(osteomyelitis, TB,** **sarcoidosis)**
* Affects **liver, kidney, spleen, adrenals** *but not heart*
* can affect multiple organs, _heart is usually spared_
34
**Hemodialysis associated Amyloidosis**
_Etiology & Effects_
## Footnote
o **Accumulation of normal protein** (beta‐2 microglobulin) in plasma
_o Deposits in bones and joint_s
**o Carpal tunnel syndrome, cervical spine pain**
o **Tongue deposits** can have **macroglossia**
35
**Amyloidosis**
_Clinical Presentations_
* **Deposition of an extracellular proteinaceous materia**l‐often **immunoglobulins**
* all types have common feature of a _β‐pleated sheet molecular configuration_
* **Macroglossia**
* **can be massive and exhibit dental indentations** (**crenation**) with _yellowish peripheral nodules_
* **Gingiva**: _usually normal in color,_ but *may be bluish, spongy and enlarged*
* **Xerostomia** _if deposits in salivary glands_
* **Mucosal petechiae** can be seen
36
Oral manifestation of which Systemic Disease?
**Amyloidosis**
Nodular “waxy” depositions in skin
**deposition on the eyelid**
37
Which systemic disease has this oral manifestation?
Amyloidosis
orange, red, yellow tinge
38
Which systemic disease has this oral manifestation?
Amyloidosis
Macroglossis and crenation of tongue (indentation near the teeth area)
_skin deposits_ on the comissure,
39
Which systemic disease has this oral manifestation?
**Amyloidosis**
_macroglossia_
40
Which systemic disease has this oral manifestation?
Amyloidosis
Submucosal amyloid deposit
41
Which systemic disease has this oral manifestation?
**Amyloidosis**
_Amyloid deposition_ on the tongue is amyloid, you have papule and nodule like area, can see the **crenation** of the tooth
42
Which systemic disease has this oral manifestation?
**Amyloidosis**
_Amyloid deposition_ with **ulceration** and **petechiae**
43
Which systemic disease has these oral manifestations?
Amyloidosis
▪ different color compared to normal tongue with amyloid
44
Amyloidosis
_Mangement_
* **Medical work‐up** *to determine* **type of amyloidosis**
* T**reat underlying disease** when possible
* **No treatment available for most types**
* **Chemo drugs** _(Colchicine, Prednisone, Melphalan, Thalidomide, Cyclophosphamide)_ for **multiple myeloma**
* **Serum electrophoresis** – monoclonal gammopathy very complicated and time consuming treatment
* **Renal transplant** for _dialysis‐associated type_
* *Death* **due to cardiac failure, arrhythmias or renal failure** *_is not uncommon_* within a few year of dx
45
Diabetes Mellitus
Endocrine Disease
* a group of metabolic disorders with one common manifestation**: hyperglycemia**
* Basic problem is either _a decreased production of insulin_ or _tissue resistance to insulin_
46
Diabetes Mellitus
_Pathophysiology_
▪ **Insulin** is hormone produced by ***beta cells of the pancreatic islets of Langerhans***
▪ It is _required for the uptake of glucose by body cells_
▪ **Insulin binds specific receptors which trigger** the _intracellular events necessary for glucose uptake_
47
Diabetes Mellitus
Types
Type 1
Type 2
48
Diabetes Mellitus
Type I
_Definition_
_Demographics_
_Symptoms_
_Etiology_
_Definition_
insulin‐dependent diabetes mellitus (IDDM)
_Demographics_
5‐10% of cases
**_Juvenile onset (avg age 14)_**
_Symptoms_
* Severe **absolute lack of insulin**
* **Hyperglycemia** and **ketoacidosis**
* **Blood glucose levels of 200‐400 mg/dl** (70‐120 normal)
* **Ketoacidosis** from using *protein* and *fat* for energy instead of glucose body _can’t use glucose_
* **Thin body habitus**
_Etiology_
* **Autoimmune disease**
* Thought to be possible viral infection as trigger to Islet cell antibody destruction of beta cells
49
**Diabetes Mellitus**
Type II
_Definition_
_Demographics_
_Symptoms_
_Etiology_
_Definition_
* non‐insulin‐dependent diabetes mellitus (NIDDM)
_Demographics_
* **About 90% of cases**
* Onset in **older, obese adults (80‐90%)**; _ketoacidosis is rare_
_Symptoms_
* _Patients produce some insuli_n, can typically be treated with oral medication
* **“insulin resistance”**‐ insulin levels appear WNL or elevated
_Etiology_
* **Decreased number of insulin receptors or defective receptors**
* *Genetic abnormalities, multifactorial*
* _Growing percent_ of the **US population** as well as around the world
50
**Diabetes Mellitus**
_Complications_
▪ **Decreased neutrophil chemotaxis** ►_do not fight off infections as well as
you should_
▪ **Peripheral vascular disease**►microangiopathy
o Results in **ischemia**: kidney failure, gangrenous complications of lower limbs, retinal involvement leading to blindness
o Amputations, CVA or MI
o **Ketoacidosis** may lead to *_diabetic coma_*
51
**Diabetes Mellitus**
_Oral Findings_
**_Most often associated with Type I_** *but may be seen with Type II*
* **Periodontal disease**‐ more frequent occurrence, more rapid progression
* **Poor healing** _post oral surgery/extractions_
* **Enlargement and erythema of the attached gingiva**
* **Increased risk of infections**
* Candidiasis
* **Xerostomia**‐ 1/3 of pts complain of dryness
* **Diabetic Sialadenosi**s‐ both type I and type II
* **Mucormycosis**‐ in uncontrolled disease and the tissue becomes necrotic because it is not getting any blood supply
* **Dental Caries**o **Benign migratory glossitis**
* Increased prevalence in type I
52
**Diabetes Mellitus**
**TYPE I**
_Management_
* **Insulin injections**
* **Insulin shock**‐ _if blood glucose falls *below 40 mg/d*l_
* Treat with **dextrose**
53
**Diabetes Mellitus**
**TYPE II**
_Management_
* **Dietary modification** and **weight loss**
* **Oral hypoglycemic agents**
* ex. tolbutamide, glyburide, metformin
* *Drugs may cause* **a lichenoid drug reaction**
54
Oral Manifestation of which systemic disease
**Diabetes Mellitus**
Gingivitis = puffy red papillae here between the
central and lateral incisors
55
Oral Manifestation of which systemic disease ?
**Diabetes mellitus**
Anterior papillae are very puffy and red and fill of pus
Posterior gingiva are very
hyperplastic
56
Oral Manifestation of which systemic disease ?
Hyperplastic gingiva
57
Oral Manifestation of which systemic disease ?
Diabetes Mellitus
Sialadenosis
58
Oral Manifestation of which systemic disease ?
Diabetes Mellitus
diabetic patient who
developed *Mucormycosis*
Notice it is causing _necrosis_ in the palate
59
Which systemic disease has this oral manifestation?
**Lichenoid mucositis looks like lichen
planus**
-same reticular white pattern, but there are
areas of erosion and some ulceration as
well
-some diabetic medications can lead to this
60
Hyperthyroidism
what is its most common form?
**Graves’ Disease is the most common form**
o An autoimmune disease attacks thyroid (TSH receptor)
o Leads to _elevated release of thyroxine_
o Most often a diffuse thyroid enlargement **(goiter)**
61
Benign and malignant tumors of thyroid and pituitary gland can cause which systemic disease
**hyperthyroidism**
62
**In Hyperthyroidism, what happens to**
**T4** (thyroxine) and **TSH** values
* **Serum T4** (thyroxine) is **_elevated_**
and
* **TSH is _decreased_**
63
Hyperthyroidism
_Demographics_
▪ **5‐10X** more common in **females** ‐ 2% of women
▪ **3rd ‐ 4th** _decade of life_
64
Hyperthyroidism
What patients might complain of?
What are the symptoms?
**What patients might complain of?**
* nervousness
* heart palpitations
* heat intolerance
* muscle weakness
* emotionally labile
**What are the symptoms?**
* weight loss, excessive perspiration, tachycardia,
tremors, eyelid retraction and exophthalmos
* **20‐40%** have **ocular involvement**
o Early in the disease‐ **characteristic stare with eyelid retraction and lid lag**
o **Exophthalmos or proptosis**
▪ **Oral findings** in children can include **early eruption of teeth**
65
**Hyperthyroidism**
_Treatment_
* Treatment includes:
* **Surgery** – complete or partial removal of thyroid gland
* **Medications**
* _Propylthiouracil_ and _methimazole_ block normal use of iodine by thyroid gland
* **Radioactive iodine 131I**
* Treatment often results in **hypothyroidism**
66
Which systemic disease has this oral manifestation?
**Hyperthyroidism**
* enlargement of the neck
* characteristic stare
67
**Hypothyroidism**
What happens in Hypothyroidism?
What it is called in children & adults?
How it is diagnosed?
_What happens in Hypothyroidism?_
* Decreased levels of thyroid hormone
_What it is called in children & adults?_
* **cretinism** in children
* **myxedema** in adults
_How it is diagnosed?_
* Diagnosed by **measuring T4** (free thyroxine) in serum
68
Hypothyroidism
Primary and secondary etiologies and T4,TSH profile?
**▪ Primary Hypothyroidism**
‐ due to abnormality in thyroid gland
o **T4** *_low_*, **TSH** *_elevated_*
**▪ Secondary Hypothyroidism (less common)**
‐ pituitary gland doesn’t produce adequate
amounts of TSH
o **T4** *_low_*, **TSH** *_low_* or borderline
69
**Hashimoto’s thyroiditis** or **thyroid surgery** are main causes in adults of which systemic disease?
**Hypothyroidism**
70
Hypothyroidism
_Symptoms_
_Oral findings_
_Symptoms_
* Symptoms include lethargy, dry skin, thinning hair, swelling (edema) of face and extremities, huskiness of voice, weakness and fatigue
* ▪ Infants have failure to thrive
_Symptoms_
o **Thickened lips** and **macroglossia** due to _accumulation of glycosaminoglycans (GAGs)_
o In children can see **failure of tooth eruption** even though teeth have normal development (enamel pitting can be seen)
71
**Hypothyroidism**
_Treatment_
▪ Treatment is **thyroid hormone replacement**
▪ Prognosis is **generally good**
▪ If children are not treated in a timely fashion ► **permanent CNS damage can occur (mental retardation)**
72
Which systemic disease has this oral manifestation?
hypothyroidism
woman who had
hypothyroidism, lips are thickened, thick creases in the face
73
Which systemic disease has this oral manifestation?
hypothyroidism,
in child, still has
deciduous teeth even though its an older child
Radiographically we see the teeth have not erupted in the oral cavity
74
Which systemic disease has this oral manifestation?
**Hypothyroidism**
**Macroglossia** and **crenation** (scalloping)
of the lateral tongue
75
Before and after tx of which systemic disease?
hypothyroidism
76
**Hyperparathyroidism**
Can be *Primary* or *Secondary*
* Reduced amounts of PTH
* Parathormone normally stimulates osteoclasts in bone and resorption in kidney, to bring serum levels of calcium back to normal with decreased parathyroid function, serum calcium levels drop → hypocalcemia
* Lab findings ‐ PTH ↓, calcium ↓, phosphate↑, and normal renal function
* Postive Chvostek’s sign ( sign of low Calcium)
Females 2 to 4 times more than males
77
**Primary Hyperparathyroidism**
Charcterstics
o _Uncontrolled_ PTH production
o 80‐90% caused by **parathyroid adenoma**
o ~15% caused by hyperplasia
o ~ 2% caused by parathyroid carcinoma
78
**Secondary Hyperthirydoism**
_Charcterstics_
o PTH continuously produced in response to chronic low serum calcium (usually associated with chronic renal disease)
79
**Hyperparathyroidism**
_Classic triad_
_of_
**bones, stones, and groans (& moans)**
* **Bones** – Changes in the bones:
* Subperiosteal resorption of distal phalanges (early in disease)
* Loss of lamina dura around roots (early in disease)
* Loss/blurring of trabecular density in bone with resultant “ground glass” appearance in radiographs
* Brown tumor
* **Stones** – renal calculi (especially with primary disease) due to elevated serum calcium basically kidney stones
* **Groans** – duodenal ulcers
* **Moans** – changes in mental status mild dementia
80
What are the manifestation of **_Brown tumor_** in Hyperparathyroidism
* **uni‐ or multilocular Radiolucency** (pelvis, ribs, mandible)
* seen with _persistent disease_
* histology of **giant cell lesion** (**like CGCG**)
81
What is the most severe bone manifestation in people with hyperparathyroidism
**Steitis fibrosa cystica**
Where we see **central degeneration** and **fibrosis** of **longstanding brown tumors**
82
**Hyperparathyroidism**
_Bones Manfestiation_
* **Subperiosteal resorption** of distal phalanges (early in disease)
* **Loss of lamina dura** around roots (early in disease)
* **Loss/blurring of trabecular density** _in bone with resultant_
* **“ground glass”** appearance in radiographs
* **Brown tumor**
* **Steitis fibrosa cystica**
83
Hyperparathyroidism
Treatment
It is typically **surgical removal** of _a portion or all of the
parathyroid glands_
84
**Hypoparathyroidism**
_Etiology_
▪ Can be due to **inadvertent surgical removal** when **thyroid gland** is _excised_ or to _autoimmine destruction._
▪ **DiGeorge syndrome** (anomaly) and **endocrine‐candidiasis syndrome** can show this.
85
What is _a Chvostek’s sign?_
* The Chvostek sign is a clinical finding associated with hypocalcemia, or low levels of calcium in the blood.
* This clinical sign refers to a twitch of the facial muscles that occurs when gently tapping an individual's when the facial nerve below the zygomatic process
86
**Hypoparathyroidism**
in young children
* If develops in young children **tooth development can be affected**
* **_pitting enamel hypoplasia_** and **_failure of eruption_**
* **Persistent oral candidiasis** in a young patient may be a sign of the onset of **endocrine‐candidiasis syndrome** *(check for other endocrine abnormalities)*
87
An Oral Manifestaion of which systemic disease?
Hyperparathyroidism
in young children
88
**Hyperparathyroidism**
_Management_
* Oral vitamin D precursor
* vitamin D2 (or ergocalciferol)
* Dietary supplements of calcium
* Teriparatide (a portion of PTH) injections twice daily
89
**Pseudohypoparathyroidism**
Also known as?
_What is it?_
_How it appears clinically_
_What is its lab findings?_
_Also known as?_
Albright hereditary osteodystrophy
_What is it?_
▪ **Normal parathyroid** and **PTH**, but activation of target cells is **dysfunctional**
_How it appears clinically?_
▪ Clinically, patient appears to have hypoparathyroidism
o Based on **elevated serum levels of PTH seen with hypocalcemia,hyperphosphatemia** and **normal renal function**
Lab findings
**PTH ↑, calcium ↓, phosphate** ↑
90
**Pseudohypoparathyroidism**
Types
What causes it
Type of inheritance & mutation
*_Types_*
Two broad disorders multiple subtypes
**Type I and Type II**
*_What causes it_*
**Abnormal biochemical pathway**s that result in lack of target cell activation despite **normal levels of PTH**
*_Type of inheritance_*
Can be _autosomal dominant inheritance_ –
**defective PTH** **receptor on the target cells**
91
**Pseudohypoparathyroidism**
_Management_
- Vitamin D and calcium supplements
- Serum and urinary calcium are monitored
92
**Pseudohypoparathyroidism**
_Oral Findings_
* *- Generalized enamel hypoplasia**
- **Widened pulp chambers** with **pulpal calcifications (“dagger” shaped pulp stones)**
- _Oligodontia_
- _Delayed eruption_
- _Blunting of root apices_
93
Which systemic disease has this oral manifestation?
**Pseudohypoparathyroidism**
pulp chambers are very
elongated
94
Which systemic disease has this oral manifestation?
**Pseudohypoparathyroidism**
issues with eruption, no
pulp stones present
95
Hereditary
Hypophosphatemia
Also known as?
What type of inheritance?
What mutation causes it?
**Also known as?**
vitamin D‐resistant rickets looks like they have rickets.
**What type of inheritance?**
▪ Most cases are X‐linked inheritance (males)
**What mutation causes it?**
o Mutation in PHEX ‐ zinc metalloproteinase gene
▪ phosphate regulating gene with endopeptidase activity on
the X chromosome
▪ Mutation affects **metabolism of vitamin D precursors to the active metabolite**
o Therefore _low or no absorption of calcium_
▪ _Decreased capacity for reabsorption of phosphate from the renal tubules_
96
**Hereditary
Hypophosphatemia/vitamin D‐resistant rickets**
Clincal Findings
_Lab findings_
_Clinical Findings_
▪ Clinical features similar to those of rickets, but resistant to treatment with vitamin D
o **Short stature** (upper body fairly normal, lower body shortened)
o **Lower limbs short and bowed**
▪ _Similar to Vitamin D‐dependent rickets except no hypocalcification of teeth_
▪ **Teeth have enlarged pulp chambers and elongated pulp horns** (extend to DEJ)
▪ With **minor attrition of occlusal cusps, pulp is exposed to the oral cavity**
▪ Exposures are often very small and when note periapical radiolucencies, it appears that otherwise normal teeth have periapical pathology
_Lab findings:_
**↓ serum phosphate**
97
What systemic disease causes this oral symptoms?
Hereditary
Hypophosphatemia/vitamin D‐resistant rickets
teeth look fairly
normal, have a draining abscess with ulcers and perilous
98
What systemic disease causes this oral symptoms?
Hereditary
Hypophosphatemia/vitamin D‐resistant rickets
teeth look fairly
normal, have a draining abscess with ulcers and perilous
99
**Hereditary
Hypophosphatemia**
_Histology_
Enlarged pulp horns
o Can extend up to DEJ
Abnormal globular dentin
o Dentin may exhibit clefting
Enamel clefts
Bacteria noted in enamel, dentin and pulp
o Pulpal involvement leads to necrosis and development of the periapical pathology
100
Hypophosphatasia
What is it?
Type of inheritance?
Clincal findings?
Lab Findings?
**What is it?**
Rare metabolic bone disease
**Type of inheritance:**
**_Autosomal recessive_** inheritance, generally the younger the age of
onset the more severe the expression
**Clincal findings:**
Bone abnormalities resemble rickets
Often presents with premature loss of primary teeth
o not from periodontal disease, thought that marked reduction, or lack, of cementum allows exfoliation
**Lab findings:**
↓ alkaline phosphatase, ↑ blood and urinary
phosphoethanolamine
101
Hypophosphatasia
Types
4
Perinatal
Infantile
Childhood
Adult
102
Hypophosphatasia
_Perinatal_
o Most severe manifestations
o Death in a few hours secondary to respiratory failure
o Marked hypocalcification of skeleton
103
Hypophosphatasia
_Infantile_
o Diagnosed ~4‐6 months due to failure to grow
o Skeletal malformations
shortened bowed limbs, rib and skull abnormalities
o Nephrocalcinosis, nephrolithiasis
Can have premature exfoliation of teeth
104
Hypophosphatasia
_Childhood_
o Diagnosed at later age, variable clinical expression
o Early sign is **premature loss of primary teeth, may be the only teeth affected**
o **Large pulp chambers** *and* **alveolar bone loss**
o P**remature fusion of cranial fontanels** can lead to _increase
intracranial pressure and brain damage_
105
Hypophosphatasia
_Adult_
o Mild presentation
o **Premature loss of primary or permanent dentition**; patient may be **edentulous**
o **Stress fractures in _feet_**
o Increased number of fractures assoc. with minor trauma
106
**Acromegaly**
vs
**Gigantism**
**Acromegaly** – excess production of growth hormone **after** closure of the epiphyseal plates
**Gigantism** – excess production of growth hormone **before**
closure of the epiphyseal plates
107
Acromegaly
Etiology
Clinical signs
Oral Findings
Etiology
▪ Usually due to _a pituitary adenoma_
Clinical signs
▪ _Renewed growth of small bones_ of the hands and feet as well as membranous bones of the skull and jaws
▪ Soft tissues are also affected
Oral Findings
▪ Mandibular ***prognathism***, ***macroglossia***, and **diastema formation**
108
Which systemic disease has these clinical manifestations?
**Acromegaly**
109
_Addison’s Disease_
**also known as**
**etiology**
**when do clinical symptoms appear?**
**also known as**
Hypoadrenocorticism
**etiology**
```
Insufficient production of corticosteroid hormones due to destruction of
adrenal cortex (autoimmune, infection, tumors, etc.)
```
**when do clinical symptoms appear?**
▪ Need ~90% destruction of gland before clinical symptoms
110
Addison’s Disease /Hypoadrenocorticism
Clincal symptoms
Lab findings
_Clincal symptoms_
* diffuse hyperpigmentation of skin (bronzing), fatigue,
* irritability, depression, weakness, etc.
* Oral mucosa shows **diffuse** *or* **patchy macular pigmentations flattened pigmentations**
_Lab findings_
failure of cortisol to rise in response to a rapid ACTH stimulation
111
Which systemic disease manfiest like this?
**Addison’s Disease**
112
Which systemic disease manfiest like this?
**Addison’s Disease**
113
**Vitamin B3 (Niacin)**
_Deficiency known as_
_Classid Triad_
_Oral symptoms_
**Deficiency**
pellagra
**Classic triad**
**D**ermatitis, **D**ementia, **D**iarrhea
**Oral symptoms**
_stomatitis_ and _glossitis_
114
Which systemic disease is associated with this symptom?
_Pellagra_
Deficience in Vitamine B3 (Niacin)
115
Pellagra
Deficience in Vitamine B3 (Niacin)
Dermititis of the skin
116
Which systemic disease manifests like this?
Pellagra
Deficience in Vitamine B3 (Niacin)
erythema of the tongue
117
**Vitamin C**
**Deficiency**
_Known as_
scurvy
118
**Iron Deficiency
Anemia**
_Charcterstics of the_ Anemia
_Symptoms_
**Charctersics of Anemia**
a _hypochromic_, _microcytic_ anemia
*decrease in color, smaller
than normal*
**Symptoms**
fatigue, light headedness, lack of energy
.
119
Iron Deficiency
Anemia
Oral Symptoms
* **Angular cheilitis** (corner of the mouth ulceration)
* **Dysphagia** (difficulty swallowing)
* **Atrophic glossitis** (bald tongue, loss of papilla) beefy colored tongue
* **Glossodynia** (burning tongue)
120
Iron Deficiency
Anemia
_Treatment_
Treated with iron supplements, extreme cases with blood infusions
121
Plummer‐Vinson
Syndrome
What is it ( assiosited with what difficiency)?
Demographics?
**What is it ( assiosited with what difficiency)?**
* Iron deficiency anemia with **glossitis** and **esophageal strictures**
* _Similar signs and symptoms_ as iron deficiency
**Demographics**
* Mostly women 30‐50 years old
* Northern European heritage more common
122
**Plummer‐Vinson
Syndrome**
Why it is a concern?
**Why it is a concern?**
Premalignant process
o ↑ incidence of oral and esophageal SCCa
123
**Plummer‐Vinson
Syndrome**
*Iron Deficiency
Anemia*
_Treatment_
* Treated with iron supplements
* Need long term follow up for eval of SCCa
124
Which systemic diseaswe associated with these oral manifestations?
**PLUMMER‐VINSON
SYNDROME**
denuded tongue
and angular chelitis
125
Which systemic diseaswe associated with these oral manifestations?
_PLUMMER‐VINSON
SYNDROME_
**Angular chelitis** (top) hard to get rid of them
**Atrophic Glossitis** (bottom) red beefy tongue
126
How blood looks with
**Top photo**: in Iron Deficiency Anemia, Plummer‐Vinson Syndrome, small area that is pale in the center
**Bottom photo** : normal smear, center is not pale
127
**Pernicious Anemia**
Type of Anemia?
What difficiency?
Causes?
_Type of Anemia?_
**_Megaloblastic_** Anemia
_What difficiency?_
**Vitamin B12 difficiency**
_Causes?_
* Poor absorption of vitamin B12 (extrinsic factor, cobalamin)
* These patients _lack intrinsic factor,_ usually **due to autoimmune destruction of parietal cells**
* ***Intrinsic factor*** produced by parietal cells in the stomach is **needed for absorption of B12**
128
Pernicious Anemia
Symptoms
* Have **burning sensation of** *tongue, lips and buccal mucosa*
* One of the things you need to rule out in patients with burning mouth syndrome
* **50‐60% of patients** have **tongue symptoms,** see **atrophy** (papilla denuded) and **erythema**
129
Pernicious Anemia
_Treatment_
* monthly IM injections of **cyanocobalamin**
* **cannot take **_**B12 orally**, you need injections_
130
Smear blood of Pernicious Anemia vs normal blood smear
not biconcave
131
**Pernicious Anemia**
CLASSIC TRIAD
1 – **Generalized weakness**
2 – **Painful tongue**
3 – **Numbness or tingling of the
extremities**
132
Which systemic disease manifests like this?
**Pernicious Anemia**
glossitis, denuded papillae
133
This is a before and after of which systemic disease?
**Pernicious Anemia**
## Footnote
denuded tongue and then the papillae is back again after the treatment. You have to get injections for the rest of your life
134
**UREMIC STOMATITIS**
_What is it?_
_What causes the oral lesions?_
What is it?
* Uncommon complication of renal failure
* Patients have markedly **elevated levels of urea** _in their blood stream_
**What causes the oral lesions?**
* Cause of oral lesions is unclear, may be urease produced by normal flora degrades urea in saliva liberating free ammonia which damages mucosa
135
**UREMIC STOMATITIS**
_Clinical Presentation_
Most cases in patients with **acute renal failure**
▪ Abrupt onset of **white plaques or crusts**
o _Bad taste_ and _burning_ also possible
o **May be** *painful*
▪ Usually on the **buccal mucosa, tongue and floor of mouth**
o If localized to the tongue can mimic ***oral hairy leukoplakia***
▪ May detect **an odor of ammonia or urine** on the patients breath
136
Which systemic disease has this oral manifestation?
Uremic Stomatitis
137
**Uremic Stomatitis**
_Treatment_
▪ Usually clears within a few days _after renal dialysis has begun_
▪ **Mildly acidic mouth rinses** seem to clear oral lesions *(ex. diluted hydrogen peroxide)*
▪ **Palliative treatment *_for pain_*** includes _ice chips or a topical anesthetic_
138
Before and after treatment of which systemic disease?
**Uremic Stomatitis**
Before and
After Tx with Dialysis
changes on ventral and lateral side
of the tongue, better outcome after dialysis
139
Reiter’s Syndrome
also known as?
Corrlate with which antigen?
Associated with what?
_also known as?_
**Reactive arthritis**
_Corrlate with which antigen?_
Correlation with **HLA B27 (\> 70%)**
_Associated with what?_
Typically seen after patient has either **a bacterial dysentery or an STD** sometimes **chlamydia**
140
**Reiter’s Syndrome (Reactive Arthritis)**
_Etiology_
_Demographic_
_Clinical Presentation_
_Etiology_
* Thought to be due to **an abnormal immune response to the infection**
_Demographic_
* Almost **exclusively** seen ***in males*** _in their twenties_
_Clinical Presentation_
* Oral lesions (~ 20% of cases) include **painless oral ulcerations**, **erosions/erythema of mucosa**, as well as erosions of the tongue which can mimic geographic tongue (both clinically and histologically)
141
Which systemic disease has these oral manifestations?
Reiter’s Syndrome (Reactive arthritis)
This not actually a geogrpahic tongue!
142
Which systemic disease has these oral manifestations?
Reiter’s Syndrome (Reactive arthritis)
Top: erythema on the palate and
areas of ulceration
Bottom: classic look of geographic
tongue, but it is not geo tongue. They are symptoms of Reiter’s
143
**Reiter’s Syndrome**
Classic Triad
Reiter’s Syndrome
**Classic Triad**
▪ 1 – Polyarthritis (lasting more than one month)
▪ 2 – Conjunctivitis or uveitis
▪ 3 – Urethritis
144
**Infective endocarditis**
▪ Janeway lesions
o Seen on palms of hands and soles of feet (tiny micro emboli that are causing lesions on the hands and feet)
▪ **Erythematous macule or petechiae**
▪ _Painless_
▪ **Septic micro emboli**
145
Which systemic disease has these clinical manifestations
Infective Endocardiatios
Janeway lesions
These are **Septic Emboli**
146
**Hyperparathyroidism**
_What is it?_
_What are its types?_
_What is it?_
* **Excess of circulating parathyroid hormone important in formation of osseous structures**
* Increase bone remodeling but tips the balance of osteoblastic and osteoclastic activity in favor or osteoclastic resorption
* it's a systemic endocrine disease
_What are its types?_
**o Primary hyperparathyroidism Type** 1
▪ Uncontrolled parathyroid hormone production by
parathyroid neoplasm (adenoma \>\> hyperplasia \>
adenocarcinoma)
**o Secondary hyperparathyroidism Type 2**
▪ Continuous parathyroid hormone production in
response to chronic low serum calcium levels
▪ Renal osteodystrophy
147
Which systemic disease is this?
Hyperparathyroidism
Here we see we see
* _a granular appearance of the max and mand bone everywhere,_ it is **not localized**.
* There is a **loss of bone density** and **the loss of definition of cortical bone.**
* Here we see a **loss of definition of lamina dura** as well _because it is now granular, and is not as clear._
148
Which systemic disease shows radiographically like this ?
**Hyperparathyroidism**; this is the **brown tumor** which is sometimes **well or ill defined,** **multi or unilocular radiolucency** with **granular septation.**
If you have a patient that is younger than 15-20 years old that has **a central giant cell granuloma** ► you have to check that patient for hyperparathyroidism, because it could be a brown tumor.
149
Which systemic disease shows radiographically like this ?
This is another medical CT scan. You see the granular appearance of
the maxilla, skull, and well-defined multilocular radiolucency with
granulation. We call this a brown tumor because it is associated with
**hyperparathyroidism.**
**( s**ystemic endocrine diseases)
150
Which systemic disease shows radiographically like this ?
Hyperparathyroidism
## Footnote
On our intraoral radiographs, we see **loss of definition of lamina dura** because the bone now has **a granular appearance** which extends to the lamina dura.
The teeth are usually normal, but there is a loss of lamina
dura around the teeth. These teeth are not mobile.
151
Which systemic disease shows radiographically like this?
a medical CT scan of a patient with **secondary hyperparathyroidism.**
We see a **lack of cortical bone** – no normal cortical bone. Inside the skull
we have **a granular appearance**, with **radiolucent and radiopaque dots**, we call this **a salt and pepper dots**.
This is why we call this a salt and pepper appearance, _there is no normal cortical bone._
*( systemic endocrine diseases)*
152
**Hyperparathyroidism**
Radiographic features
▪ **Stones, bones, moans, and groans**
▪ **Radiolucent appearance** (_generalized osteopenia_) What we see in the bones is one of the earliest radiographic features that we see = sudden erosions in the phalanges in the bones of the hands followed by mineralization of the skeleton including max and mand, skull base. As a
result, you have generalized osteopenia. **10% of these patients have brown tumors**
▪ **Brown tumors:** Brown tumors are the same as central giant cell granulomas. When they are associated with hyperparathyroidism, we call them brown tumors.
▪ **Punctate or nodular calcifications** in the joints and kidneys =stones
▪ **Entire calvaria** has _a granular appearance_ classically known as the **“salt and pepper”** skull as a result of the generalized osteopenia, we have this granular appearance in the skull
153
**Hypoparathyroidism**
_Radiographic features_
What is Hypoparathyroidism:
## Footnote
_Insufficient secretion of PTH
o Damage or removal of the parathyroid glands during thyroid surgery_
Principal radiographic change: calcification of the basal ganglia
Sometimes we have **pseudohypoparathyroidism.** This happens when we have a normal amount of PTH, but there is something wrong with the response of the target tissue. So, we have normal PTH but abnormal response to PTH.
**dental enamel hypoplasia, external root
resorption, delayed eruption, or root dilaceration**
154
Which systemic disease shows radiographically like this?
we have 2 Pas of patients
**with pseudohypoparathyroidism**.
* hypoplasia of enamel, tooth material
* hypoplastic tooth bud ( hypoplastic means arrested development)
* delayed eruption,
* external root resorption.
155
Hyperpituitarism
What is it?
What is its types
_What is it?_
* Hyperfunction of the anterior lobe of the pituitary gland caused by a benign functioning tumor of the anterior lobe
* it's a systemic endocrine disease
_What is its types ?_
▪ Types based _on age of onset_
* **Gigantism** happens in ***children***, **generalized overgrowth of most long bones, hard and soft tissue**. These patients are usually very tall. Proportion in these patients may be normal, but very large appearance.
* **Acromegaly** Increased hyperfunction in **adult patients**. In these patients, the epiphysis of the long bones already closed, **you can see in the mand or max.** One of the observable features is **enlargement of the whole mandible/skull/sinuses/soft tissue.** Patients usually present with _“My hat is not fitting anymore, my denture is not fitting anymore”_
156
**Hyperpituitarism**
_Radiographic
Features_
**▪ General features**
* _Enlargement_ (ballooning) of the **sella turcica**
* _Enlargement_ of the **paranasal sinuses** (especially the **frontal sinus**)
* _Thickening_ of the outer table of the **skull**
**▪ Jaws**
* _Enlargement of the **jaws**,_ most notably the **mandible**
* **Class III skeletal** as a result of the mandible enlargement with growth centering in the condylar head
**▪ Teeth and associated structures**
* _Spacing of the teeth, enlargement of tongue_, it could result in spacing of teeth in the anterior region
o **Hypercementosis the forces** are higher now
* so you may see H**_ypercementosis_**
* Hypercementosis is **excessive deposition of non-neoplastic cementum over normal root cementum,** which alters root morphology. This cementum may be either hypocellular or cellular in natur*
157
Which systemic disease shows radiographically like this?
Acromegaly (Hyperpituitarism)
* enlargement of the mandibular bone with a high degree of enlargement
* a class III appearance
* enlargement of sella tursica because of the pituitary gland enlargement
158
Hypopituitarism
What is it?
What is it called?
How it appears radiographically?
_What is it?_
Reduced secretion of pituitary hormones
it's a systemic endocrine disease
_What is it called?_
**Dwarfism**
_How it appears radiographically?_
▪ Finding of the jaws
o **Normal eruption of primary dentition** but _delayed exfoliation_
o **Small jaws -**\> crowding and malocclusion because there is not enough space for the teeth to erupt
159
Which systemic disease manifests radiographically like this?
Dwarfism
* hypopituitarism*
* We see* multiple dental anomalies: **hypodontia, radicular fusion,** fused roots of left lateral incisor and left canine **and impacted permanent teeth.**
* (from google)*
160
**Osteoporosis**
What is it?
Why it happens?
What the bone are like?
_What is it?_
▪ **_Generalized decrease in bone mass_ i**n which the histologic appearance of bone is normal, it is a metabolic bone diseases (MBD)
_Why it happens?_
* **Aging process (postmenopausal women)** bone mass usually increases until 30 years of age, and then there is a gradual decrease- about 8% loss in females and 3% loss in males
* **Nutritional deficiencies**
* **Hormonal imbalance**
* **Inactivity**
* **Corticosteroid or heparin therapy**
_What the bone are like?_
▪ **More prone to fracture** (distal radius, proximal femur, ribs, and vertebrae)
161
Which systemic disease shows radiographically like this?
_Osteoporosis_
* **reduction in bone density,**
* **larger bone marrow spaces.**
We **need more tests to confirm osteoporosis** besides dental radiographs.
162
Osteopetrosis
What is it?
How is the bone is like?
What are the effects on other structures?
What are the consquences?
_What is it?_
**Defect in the differentiation and function of osteoclast**s, (osteoclasts that are used to resorb bone in the bone modeling process. They are not functioning properly ►so we have an increase in bone density.) It is a metabolic bone diseases (MBD)
_How is the bone like?_
Bone is **dense, fragile, and _susceptible to fracture_** _and **infection ►**_we now have a_n osseous structure but small bone marrow spaces_
_What are the effects on other structures?_
It compromised vascular structures and cranial nerves
163
**Osteopetrosis**
_What are the consquences?_
* Compression of the cranial nerves as they pass through the narrowed skull foramina -\> **blindness, deafness, vestibular nerve dysfunction, and facial nerve paralysis.**
* Compromises hematopoiesis Poor vascularity -\> **osteomyelitis**
164
Which systemic disease shows radiographically like this?
medical CT scan with a patient with **osteopetrosis**- very dense. Not a
nice definition of the cortical bone. We see decreased in size of skull
foramina.
165
Which systemic disease shows radiographically like this?
pt with Osteopetrosis
we see
**Hypovascular bones** so they are *more prone to osteomyelitis*. This is a sign of sequestrum which is a sign of osteomyelitis.
166
pt with Osteopetrosis
* **Generalized increase in bone density,** increased trabeculation, loss of large bone marrow spaces.
* These patients are more prone to **osteomyelitis** _because they are Hypovascular_.
* We have to be careful in **extractions** because they don’t have the same vascularity as other healthy patients have.
* We see an **onion skin appearance** by the white arrow.
167
Rickets and
Osteomalacia
_What are they_
_Differences_
_Effects on Bones_
***_What happens?_***
They are metabolic bone diseases (MBD)
Inadequate serum and extracellular levels of ***calcium*** and ***phosphate***, ***failure of normal activity of vitamin D.***
There are different levels in the conversion from I,12- dihydroxy vitamin D that can have errors, not just
the consumption of vitamin D.
***_Differences_***
**Rickets**: disease affects the growing skeleton in i**nfants and
children**
**Osteomalacia**: disease affects the **mature skeleton in adults**
**Effects on bones:**
A softening and weakening of bones
168
In Rickets and
Osteomalacia, there is Failure of normal activity of vitamin D, what causes it?
o Lack of vitamin D in the diet
o Gastrointestinal malabsorption problems
o Lack of exposure to UV light
o Liver disease
o Kidney disease
o Defect in the intestinal target cell response
169
Clinical presentation
Ricket
vs
Osteomalacia
_Rickets_
o Growth retardation
o Short stature
o **Bowing of long bones** of the legs, waddling gait
o **Radiograph manifestations in the teeth** (especially \<3 years of age) **and jaws**
_Osteomalacia_
o Weak fragile bone structure
_o Diffuse skeletal pain_
o Susceptible to fracture with minor injury
o _Radiographic manifestations in the jaws_ **are uncommon**
o you may see **hyperplasia** *or* **thinning of mineralization of teeth. You can see hyperplasia of enamel in patients.**
170
Which systemic disease has this radiographic manifestation?
Ricket / Osteomalacia
hyperplasia or thinning of mineralization of teeth. We
can see hyperplasia of enamel in patients.
171
**Renal
Osteodystrophy**
What is it?
what can it lead to?
_What is it?_
**Chronic renal failure** produces **bone changes** by _interfering with the hydroxylation of vitamin D in the kidneys_ -\> _hypocalcemia_ -\> **inhibit the normal calcification of bone and teeth** *(it is a metabolic bone disease)*
_what can it lead to?_
(secondary hyperparathyroidism)
**level of calcium is low** leads to having **hyperparathyroidism secondary to osteodystrophy**
172
Which systemic disease manifest radiographically like this?
**Renal
Osteodystrophy 1**
*Presentation is variable*. Sometimes you see **denser or granular
appearance of bone.** You see _increase here_
but you sometimes will also see loss of definition of lamina dura, sometimes a sclerotic appearance
and trabeculation.
173
Which systemic disease manifest radiographically like this?
**Renal
Osteodystrophy 2**
sometimes you see:
* **increase in bone density**
* **loss of definition of lamina**
* **dura and cortical bone**
174
**Hypophosphatasia**
_What is it?_
_What are the types?_
**_What is it?_**
* Rare metabolic bone disease due to **lack of tissue-nonspecific alkaline phosphatase**
**_What are its types?_**
**Four types: prenatal, infantile, childhood, adult**
* _The younger the age of onset, the more severe the_
* _manifestations_
* It may have premature loss of these patients because loss of function of the lungs in these patients
175
**Hypophosphatasia**
Common factors?
Dental manfestations?
Common factors?
* _Low levels_ of **tissue-nonspecific alkaline phosphatase**
* **High** blood and urinary **phosphoethanolamine**
* **Rickets-like skeletal malformations**
Dental manfestations?
* _Premature shedding_ of **primary incisors**
* **Enamel hypoplasia**
* _Enlarged_ **pulp chambers** and **root canals**
176
Which systemic disease manifests radiographically like this?
Hypophosphatasia
177
Which systemic disease manifests radiographically like this?
large root canal structures, large root chambers, premature loss of
teeth = **hypophosphatasia.**
178
**Hypophosphatemia**
_What is it?_
_Clinical Manifestations?
Dental Manifestations?_
_What is it?_
* A **rare**, **heterogeneous** group of **inherited** metabolic disorders characterized by **decreased phosphate reabsorption** _in the distal renal tubules_
179
**Hypophosphatemia**
Clinical Manifestations?
Dental Manifestations?
_Clinical Manifestations_
* **Rickets-like skeletal malformations**
_Dental Manifestations_
* **Enlarged pulp chambers** and **root canals**
* **Periapical and periodontal abscesses** of no obvious cause
* **Enamel hypoplasia**
180
Which systemic disease manifests radiographically like this?
**Hypophosphatemia**
* Periapical lesions with radiolucency but no caries on the crown.
* There is loss of definition of cortical bone.
* On the teeth, you have:
* large pulp chambers
* hypoplasia of enamel and dentin
* periodontal and periapical lesions.
181
**Progressive Systemic
Sclerosis
(scleroderma)**
What is it?
Demographics?
_What is it?_
* **Excessive collagen deposition** resulting in **hardening (sclerosis) of the skin and other tissues** can have involvement of _GI tract, heart, kidney,etc_
_Demographics?_
* Middle aged female
182
Progressive Systemic
Sclerosis
(scleroderma)
_Clinical symptoms_
_Oral Manifestations_
_Clinical Presentation_
* Thickened, leathery quality skin
* joint pain
* exaggerated response to cold (Raynaud's disease)
* heartburn.
* more prone for heart problems and respiratory
insufficiencies,
_Oral Manifestations_
* microstomia (small mouth)
* Xerostomia (dry mouth)
* telangietasia ("spider veins")
* Increased decayed, missing and filled teeth.
* higher Gingivitis scores (usually)
* Deeper Periodontal Pockets
183
Progressive Systemic
Sclerosis
(scleroderma)
_Treatment_
Treatment for generalized symptoms may involve:
* **corticosteroids**
* **immunosuppressants, such as methotrexate or Cytoxan**
* **nonsteroidal anti-inflammatory drugs**
Depending on your symptoms, treatment can also include:
* blood pressure medication
* medication to aid breathing
* physical therapy
* light therapy, such as ultraviolet A1 phototherapy
* nitroglycerin ointment to treat localized areas of tightening of the skin
*(from google)*
184
Which systemic disease manifests radiographically like this?
Progressive Systemic
Sclerosis
(scleroderma)
## Footnote
sharp areas of resorption in the bones near muscles attached to the
angle of the mandible= masseter and medial pterygoid. You see
resorption at the coronoid process at the attachment of the temporal
bone as well.
185
Which Systemic disease manifests radiographically like this?
**Progressive Systemic
Sclerosis
(scleroderma)**
* presence of **widening of the PDL space everywhere** around the root of the tooth.
186
**Sickle Cell Anemia**
_What is it?_
_What causes it?_
_What is it?_
**Chronic hemolytic blood disorder**
_What causes it?_
▪ **Abnormal hemoglobin, resulting in anemia** -\> by increasing the production of red blood cells -\> **requires compensatory hyperplasia of the bone marrow**
187
**Sickel Cell Aniema**
Radiographics findings
* loss of this cortical bone area
* See the hair-on-end appearance on the skull
* enlargement of bone marrow spaces
* less trabeculation, more osteoporotic bone
* periapical pathology associated with teeth
with no obvious reason
188
Which systemic disease mainfest radiographically like this?
On a **sickle cell anemia** patient, you see:
* **loss of this cortical bone area**
* **the hair-on-end appearance on the skull**
189
Which systemic disease mainfest radiographically like this?
Sickel Cell Anemia
enlargement of bone marrow spaces, less trabeculation, more
osteoporotic bone. You see periapical pathology associated with teeth
with no obvious reason. You see the radioluscencies around the apex of
the mandibular teeth.
190
**Thalassemia**
What is it?
What causes it?
What is it?
* **Defect in hemoglobin synthesis**
What causes it?
* **RBC with reduced hemoglobin content** and _short life span_
191
**Thalassemia**
_Clinical and Radiographic findings_
* **Hyperplasia of the bone marrow** **component** of the bone which results _in fewer trabeculae per unit area and can **change the overall shape of the bone**_
* **Protrusive premaxilla**
* **Radiographic appearance** very similar to _Sickle Cell Anemia_
192
Which systemic disease mainfest radiographically like this?
**Thalassemia**
* **osteopenic bone (***loss bone mass and bones get weaker*)
* **radiolucent appearance of bone**
* thinning of cortical bone around the mandible and maxilla.
* Usually there is **hypoplasia of the paranasal sinuses.**
193
Suspect systemic endocrine and metabolic disease if radiographs show what?
generalized decrease in bone density of jaws (thin cortices; granular cancellous bone; loss of lamina dura).
* Refer to physician for lab tests to make the diagnosis
194
We can include certain systemic endocrine and metabolic diseases as radiographic differentials in the presence of:
o **Localized focus of radiolucency**- think what?
o **Mandibular prognathism and incisor flaring**- think what?
**o Premature shedding of primary incisors**-think what?
o **Enamel hypoplasia, enlarged pulp chambers and periapical abscesses of no obvious cau**se- think what?
o Localized focus of radiolucency► **think brown tumor and
hyperparathyroidism**
o Mandibular prognathism and incisor flaring► **think acromegaly**
o Premature shedding of primary incisors► **think
hypophosphatasia**
o Enamel hypoplasia, enlarged pulp chambers and periapical
abscesses of no obvious cause► **think hypophosphatemia**
