Oral Immunology A4: Oral Manifestations Of Autoimmune Diseases

Front 1

What two autoimmune diseases are the primary cause of mucosal vesicles?

Back 1

Pemphigoid, pemphigus

Front 2

1) What is the main oral symptom of pernicious anemia?
2) What causes pernicious anemia?
3) Is pernicious anemia macrocytic anemia or microcytic anemia?
4) What do RBC look like pernicious anemia?
5) What is the tx for pernicious anemia?
6) What cells secrete intrinsic factor?

Back 2

1) Glossitis
2) Autoantibodies to intrinsic factor - vitamin B12 deficiency
3) Macrocytic
4) Considerably enlarged - not nucleated.
5) INJECTIONS of vitamin B12 - can't give orally because it won't be absorbed
6) Parietal cells

Front 3

What is an important cause of glossitis?

Back 3

Pernicious anemia

Front 4

1) What is the main cause of Addison's disease?
2) What are the symptoms of Addison's disease?
3) Can Addison's disease be an acute disease or is it long onset?
4) What is tx?

Back 4

1) Antibodies are made to adrenal gland, causing adrenal insufficiency/failure, low steroid output
2) **Mucosal and skin pigmentation, hypotension, shock**, fatigue, weakness, anorexia, vomiting, diarrhea
3) Can occur very acutely
4) Steroid replacement therapy

Front 5

1) What is the primary manifestation of Type I diabetes mellitus in the oral cavity? How does it indicate the severity of the disease?
2) What is another manifestation? Is it linked to perio disease?

Back 5

1) Perio disease. More severe diabetes = more severe perio disease.
2) Hyperplastic gingivitis - SEPARATE from periodontal disease

Front 6

What is hyperplastic gingivitis indicative of?

Back 6

Uncontrolled diabetes.

Front 7

1) What is the main oral symptom of Sjogren's syndrome?
2) What causes Sjogren's syndrome? What parts of the body are affected?
3) What will you see in a cross section of a labial minor salivary gland?
4) What is the main gland usually affected?

Back 7

1) Salivary gland enlargement, xerostomia, dry eyes
2) Antibodies to nuclear mitochondria - salivary and lachrymal glands affected
3) Lymphocytic infiltrate
4) Parotid gland

Front 8

1) What is scleroderma?
2) What causes scleroderma?
3) Symptoms of scleroderma?

Back 8

1) Inflammatory, vascular, fibrotic changes of skin and internal organs
2) Anti-collagen, anti-DNA topoisomerase 1, anti-centromere antibodies
3) Hardening of skin, autoamputation, sclerodactyly

Front 9

Systemic lupus erythematosus (SLE):

1) What is it characterized by?
2) What type of hypersensitivity reaction is this?
3) What is it pathogenic for?
4) Characterizing symptom?
5) What symptom will you see in the oral cavity?

Back 9

1) Immune complex formation.
2) Type 3 hypersensitivity reaction
3) Skin, kidneys, joints.
4) Malar (butterfly) rash
5) Atrophic erythematous lesion (oral manifestations not common in lupus)

Front 10

Lichen planus:

1) What causes it?
2) What is the result of lichen planus? What happens?
3) This can cause a small risk of what type of carcinoma?
4) What causes most of the damage?
5) What are the most frequently affected sites? What do you see?

Back 10

1) Local insult - habits, dental material, bacterial invasion
2) **T cell activation** - inflammatory lymphocytic infiltrate, destruction of basal cell layer, hyperkeratosis
3) Squamous cell carcinoma due to epithelial dysregulation and dysplasia
4) ***Cytotoxic (CD8) T cells
5) Buccal mucosa, dorsal tongue. Reticular (lacey) or confluent papules

Front 11

Bullous diseases:

1) Where does pemphigus attack?
2) Where does linear IgA disease attack?
3) Where does pemphigoid attack?
4) Where does epidermolysis bullosa acquisitia attack?

Back 11

1) Desmoglein 3 of desmosome
2) Various components of BMZ/hemidesmosome
3) Various components of BMZ/hemidesmosome
4) Type 7 collagen of anchoring fibrils (underneath basement membrane, CT underneath)

Front 12

Epidermolysis bullosa acquisita:

1) How many forms are there of the disease?
2) Is this a hereditary disease? What kind of disorder is this?
3) What causes this and where does it attack?
4) What is characterized by?

Back 12

1) 23 forms - most are hereditary
2) No - this one is not hereditary. Autoimmune disorder.
3) Antibodies to type 7 procollagen on anchoring fibrils, leading to dermal/epidermal separation
4) Spontaneous/trauma induced blister formation

Front 13

Linear IgA disease:

1) Where does this attack?
2) How frequently does this disease occur, and what usually causes it?
3) What disease does it mimic? Symptoms?
4) What will you find along the basement membrane?

Back 13

1) Attacks hemidesmosomes/components of BMZ
2) Rare, drug associated
3) Pemphigoid. Vesicles, bullae, ulcers
4) Deposits of *IgA* that react with basement membrane antigens

Front 14

Pemphigoid:

1) How common is it?
2) What are the two forms and what are the manifestations?
3) What symptoms does the disease cause?
4) What causes this disease?

Back 14

1) **Relatively common**
2) a) Mucous membrane pemphigoid (cictracial) - oral lesions, gingival lesions in 90%. Buccal mucosa, palate affected in 30%.
b) Bullous pemphigoid - skin primarily affected, oral lesions in 40%
3) **Desquamative gingivitis** - subepithelial blisters - oral, ocular, skin
4) **Autoantibodies** - to basement membrane antigens of stratified squamous epithelium (at least 6)

Front 15

What kind of things do you see in pemphigoid immunfluorescence?

Back 15

Continuous linear band of immune deposits at the basement membrane in ~90% of patients, primarily IgG and C3

Front 16

What is a symptom of bullous pemphigoid? Where can it affect?

Back 16

Sub-epithelial separation. Arm, eye.

Front 17

Pemphigus:

1) How frequent is it?
2) What is the initial manifestation?
3) Why are bullous lesions rarely seen in the oral cavity?
4) Besides the oral cavity, where can it affect?
5) What causes this disease?
6) What kind of appearance does it have in IF?

Back 17

1) **Rare**
2) **Oral lesions** - fragile intraepithelial blisters
3) They rupture early, ulcerate
4) **Skin** - fragile vesicles and bullae
5) **Autoantibodies**, IgG, to **desmoglein-3 (desmosomes of stratified squamous epithelium), responsible for cell to cell epithelium
6) Fishnet stocking

Front 18

Pemphigus:

1) What does this disease cause in the oral cavity?
2) Where else can this cause lesions?
3) Where do skin blisters enlarge? How much of the body area can it cover?
4) What can the end result be?
5) Therapy?

Back 18

1) Desquamative gingivitis, buccal and palatal lesions
2) Lesions of conjunctiva, nasal mucosa, larynx, esophagus, vagina
3) At edges - can cover significant part of body area
4) **Can be fatal**
5) Intralesional steroids, systemic (prednisone, cyclophosphamide)