Oral Manifestations of Systemic Disease-Non-endocrine

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What is another word for Jaundice?

Icterus

What is Jaundice?

Excess bilirubin in the bloodstream accumulates in yellowish discoloration of the skin and mucosa

What are the causes of bilirubin?

1. Hemolytic anemia/Sickle cell anemia

2. Liver disease

3. Bile duct obstruction

4. Cancer

5. Gilbert syndrome (glucuronosyltransferase)

What are bilirubin levels?

Levels exceeds 2mg/dl

What is amyloidosis?

A group of conditions characterized by the deposition of an extracellular, proteinaceous substance termed amyloid

What are the systemic manifestations of amyloidosis?

Death within a few years due to cardiac or renal failure

If amyloidosis is organ limited, what kind of protein is affected

AL

What are the features of organ limited amyloidosis?

1. Limited to one organ

2. Infrequent oral features

If amyloidosis is primary and myeloma associated amyloidosis, what kind of protein is affected?

AL- light chain

What are some features of primary and myeloma associated amyloidosis?

1. Older males

2. 15-20% are due to multiple myeloma

3. location: eyelid, neck, and lips

4. Oral: thick lips and macroglossia

5. Skin: smooth-surfaced, firm, waxy papules and plaques

What type of protein is affected in secondary amyloidosis?

AA- Acute phase reactant protein

What are the features of secondary amyloisosis?

1. Result of chronic inflammatory process, such as TB, sarcoidosis or osteomyelitis

2. Spares the heart but affects the liver, kidney, spleen

What protein is affected in hemodialysis-associated amyloidosis?

ABeta-2 Microglobin protein

What features do you see in hemodialysis associated amyloidosis?

1. Protein isn’t removed by dialysis

2. Eventually deposits in the bones & joints

What are some features associated with heredofamilial?

1. Uncommon

2. Autosomal dominant in Swedish, portuguese, japanese population

3. Autosomal recessive in mediterranean

4. May develop congestive heart failure, and renal failure

What protein is affected in heredofamilial amyloidosis?

None

Fissured tongue due to enlargement

Amyloidosis

Nodule on lip

Amyloidosis

Swelling on eyelids

Amyloidosis

What is Crohn’s Disease

Inflammatory and immunologically mediated condition of unknown cause

What age group do you see those affected by Crohn’s Disease

Teenagers

Where does Crohn’s Disease manifest?

Anywhere along the GI tract

What are the oral implications of Crohn’s Disease?

Oral lesions are significant because they precede the GI lesions 30% of the time

What are some symptoms people that have GI issues in Crohn’s Disease experience?

1. Abdominal cramping

2. Diarrhea

3. Pain

4. Nausea

5. Fever

What may develop from Crohn’s Disease?

Weight loss and malnutrition

What kind of oral lesions do you see in Crohn’s Disease?

1. Diffuse, nodular swellings

2. “Cobblestone appearance” of the oral mucosa

3. Linear ulcerations of the buccal vestible

4. Treatment: sulfa drug/ prednisome

5. Oral lesions clear with treatment of GI disease

What is hypophosphatasia?

1. Rare metabolic disease

2. Autosomal recessive

How can you get hypophosphastasia?

A decrease in alkaline phosphatase enzyme

What oral manifestations do you see in hypophosphatasia?

1. Lack of cementum and bone abnormalities

2. Premature loss of teeth (especially in lower incisors)

What is another name for Vitamin D-Resistant Rickets?

Hereditary Hypophosphatemia

What is Vitamin D-Resistant Rickets?

• X-linked dominant (M>F)

• Early age (infancy/ childhood)

• Normal vit D

• Low serum phosphate (hypophosphatemia)

• Low calcium

• Clinical: Short bowing lower limbs

What are some oral manifestations seen in Vitamin D-Resistant Rickets

1. Large pulp horns extending to DEJ

2. Multiple non-vital teeth

3. Absence of caries or trauma

What is Iron Deficiency Anemia?

The most common cause of anemia

What are some clinical features of Iron Deficiency Anemia?

Fatigue, tired, lightheaded, lack of energy

What are some oral features of Iron Deficiency Anemia?

1. Angular cheilitis

2. Atrophic tongue

3. Glossitis

4. Burning sensation maybe due to candidiasis

What is another name for Plummer-Vinson Syndrome?

Paterson-Kelly syndrome

What is Plummer-Vinson Syndrome (Paterson-Kelly syndrome)?

A rare condition reported in women of Scandinavian or North European background and characterized by:

1. Iron deficiency anemia

2. Dysphagia

3. Atrophic glossitis

4. Esophageal webs

5. Koilonychia (spoon like nails)

6. Premalignant and associated with oral and esophageal SCC

What is Koilonycha

Spoon like nails

Glossitis

Esophageal webs