Oral Manifestations of Systemic Disease Flashcards
What are Gastrointestinal Diseases
what are example of them?
Inflammatory bowel diseases associated with oral findings
▪ Crohn disease
▪ Ulcerative colitis
▪ Pyostomatitis vegetans
▪ Celiac Disease
Crohn Disease
Regional Ileitis
What areas does affect?
What are the symptoms?
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
Crohn Disease
Regional Ileitis
When it is diagnosed?
Etiology?
Prevalence?
What are the oral implactions?
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
Crohn Disease
Histopathology
● 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
Crohn Disease
Oral Findings
- 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
Crohn Disease
Treatment
- 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
Which systemic disease manifests like this?
Crohn Disease
- Patients can also get angular cheilitis
- Above the Linear ulceration, can see a flap like structure which is the hyperplastic margin
Which systemic disease has this oral manifestation?
- we see the ulceration and hyperplastic tissue surrounding it.
Which systemic disease has this oral manifestation?
Crohn Disease
Nodular appearance of buccal mucosa
Which systemic disease has this oral manifestation?
Crohn Disease
we see more nodules
Which systemic disease has this oral manifestation?
Crohn Disease
Linear granulomatous ulcerations
But they are not the aphthous ulcerations but the more
linear type
Ulcerative Colitis
- What are the symptoms
- What type of Cancer risk it presents?
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
Ulcerative Colitis
Oral Lesions
● 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)
Ulcerative Colitis
Management
● Use of anti‐inflammatory medications
o Sulfasalazine or Prednisone
● If medical means do not succeed► then removal of part or all of colon
How different is Ulcerative Collitis from Crohn Disease?
Unlike Crohn’s, lesions extend in a continuous fashion proximally from the rectum (no skip lesions) and histologically don’t have granulomas
What is the difference between Inflammatory Bowel
Disease IBD & Irritable
Bowel Syndrome IBS
● 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
Pseudomembranous
Ulcerative Colitis
Causes?
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
Pyostomatitis
Vegetans
what is it?
Demographics?
Symptoms onset?
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
Pyostomatitis
Vegetans
Oral Symptoms
and
Most common sites in the mouth?
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
Pyostomatitis
vegetans
Treatment
● 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
Oral Manifestations of which systemic disease?
Pyostomatitis
Vegetans
Oral Manifestations of which systemic disease?
Pyostomatitis
vegetans
Oral Manifestations of which systemic disease?
Pyostomatitis
vegetans
Snail track
appearance
Celiac (Sprue) Disease
What is it?
Which gene is involved?
Symptoms?
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
Patients with Celiac (Sprue) Disease have risk of developing which cancer?
● 10‐15% risk of GI lymphoma
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
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
Amyloidosis
what are its Types and what they are associated with?
- ▪ Primary amyloidosis is associated with multiple myeloma
- Reactive systemic amyloidosis
- Hemodialysis associated amyloidosis kidney dysfunction
- Hereditary amyloidosis (Familial Mediterranean Fever), present with polyneuropathies, cardiac arrhythmias, renal failure, CHF
- Localized dermal amyloidosis
Primary amyloidosis is associated with which cancer?
multiple myeloma
Hemodialysis associated amyloidosis leads to ——
kidney dysfunction
Amyloidosis
Organ Limited
Clinically
- Rare in oral cavity
- Amyloid nodule, asymptomatic submucosal deposit
- Not associated with systemic symptoms
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
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
Hemodialysis associated Amyloidosis
Etiology & Effects
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
Amyloidosis
Clinical Presentations
-
Deposition of an extracellular proteinaceous material‐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
Oral manifestation of which Systemic Disease?
Amyloidosis
Nodular “waxy” depositions in skin
deposition on the eyelid
Which systemic disease has this oral manifestation?
Amyloidosis
orange, red, yellow tinge
Which systemic disease has this oral manifestation?
Amyloidosis
Macroglossis and crenation of tongue (indentation near the teeth area)
skin deposits on the comissure,
Which systemic disease has this oral manifestation?
Amyloidosis
macroglossia
Which systemic disease has this oral manifestation?
Amyloidosis
Submucosal amyloid deposit
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
Which systemic disease has this oral manifestation?
Amyloidosis
Amyloid deposition with ulceration and petechiae
Which systemic disease has these oral manifestations?
Amyloidosis
▪ different color compared to normal tongue with amyloid
Amyloidosis
Mangement
- Medical work‐up to determine type of amyloidosis
- Treat 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
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
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
Diabetes Mellitus
Types
Type 1
Type 2
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
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
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
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 Sialadenosis‐ both type I and type II
- Mucormycosis‐ in uncontrolled disease and the tissue becomes necrotic because it is not getting any blood supply
-
Dental Carieso Benign migratory glossitis
- Increased prevalence in type I
Diabetes Mellitus
TYPE I
Management
- Insulin injections
-
Insulin shock‐ if blood glucose falls below 40 mg/dl
- Treat with dextrose
Diabetes Mellitus
TYPE II
Management
- Dietary modification and weight loss
-
Oral hypoglycemic agents
- ex. tolbutamide, glyburide, metformin
- Drugs may cause a lichenoid drug reaction
Oral Manifestation of which systemic disease
Diabetes Mellitus
Gingivitis = puffy red papillae here between the
central and lateral incisors
Oral Manifestation of which systemic disease ?
Diabetes mellitus
Anterior papillae are very puffy and red and fill of pus
Posterior gingiva are very
hyperplastic
Oral Manifestation of which systemic disease ?
Hyperplastic gingiva
Oral Manifestation of which systemic disease ?
Diabetes Mellitus
Sialadenosis
Oral Manifestation of which systemic disease ?
Diabetes Mellitus
diabetic patient who
developed Mucormycosis
Notice it is causing necrosis in the palate
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
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)
Benign and malignant tumors of thyroid and pituitary gland can cause which systemic disease
hyperthyroidism
In Hyperthyroidism, what happens to
T4 (thyroxine) and TSH values
- Serum T4 (thyroxine) is elevated
and
- TSH is decreased
Hyperthyroidism
Demographics
▪ 5‐10X more common in females ‐ 2% of women
▪ 3rd ‐ 4th decade of life
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
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
Which systemic disease has this oral manifestation?
Hyperthyroidism
- enlargement of the neck
- characteristic stare
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
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
Hashimoto’s thyroiditis or thyroid surgery are main causes in adults of which systemic disease?
Hypothyroidism
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)
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)
Which systemic disease has this oral manifestation?
hypothyroidism
woman who had
hypothyroidism, lips are thickened, thick creases in the face
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
Which systemic disease has this oral manifestation?
Hypothyroidism
Macroglossia and crenation (scalloping)
of the lateral tongue
Before and after tx of which systemic disease?
hypothyroidism
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
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