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Chapter 3
Immunity
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1

Outline
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
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The Acquired Immune Response
Antigens
Cellular Involvement in the Immune Response
Major Divisions of the Immune Response
 Memory and Immunity
 Types of Immunity
 Immunopathology
 Oral Diseases with Immunologic Pathogenesis
 Autoimmune Diseases that Affect the Oral
Cavity
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The Acquired Immune Response


(pg. 82)
Defends the body against injury



Has the capacity to remember and respond
more quickly the second time a foreign
material enters the body
Works with the inflammatory response and a
working repair process
Involves white blood cells, especially
lymphocytes and their products
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Antigens
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
Foreign substances


(pg. 82)
Mainly proteins, often microorganisms and
their toxins
Human cells that have been transformed

May be tumor cells, or cells infected with
viruses
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Antigens (cont.)
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Human tissue
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
Organ transplants, tissue grafts, incompatible
blood types during a transfusion
Autoimmune diseases

Tissue from the person’s own body becomes
an antigen
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Cellular Involvement in the
Immune Response
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B lymphocytes
T lymphocytes
Macrophages
Cytokines
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Cellular Involvement in the
Immune Response (cont.)


(pg. 83)
Cells that are able to recognize and
respond to antigen

Derived from precursor cells in bone marrow
(stem cells)
 20% to 25% of the WBC (white blood cell)
population

Two main types



B lymphocytes (B cells)
T lymphocytes (T cells)
Also there are natural killer cells (NK cells)

Can destroy cells recognized as foreign
without recognizing specific antigens
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Cellular Involvement in the
Immune Response (cont.)
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B Lymphocytes


Mature and reside in lymphoid tissue


(pgs. 83-84)
Lymph nodes, tonsils, and other body tissue
B lymphocytes travel to the site of injury
when stimulated by antigen

Two main types
• Plasma cells

Produce specific antibodies

Retains the memory of previously encountered
antigen and will clone itself in the presence of antigen
• B memory cell
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B Lymphocytes (cont.)
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Plasma Cells

Produce antibodies that are categorized
into 5 classes of immunoglobulins, which
are carried in blood serum



All have the same basic “Y” structure, but have
an area with variable (V) structure at the tips of
the Y
The stem of the Y is constant (C) for all 5
types, and links the antibody to other
components of the immune response
Immune complex

Antigen combined with antibody
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T Lymphocytes
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Travel to the thymus and mature
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(pgs. 83, 85)
The thymus is large in an infant, shrinks as the
child matures
Several different types of T lymphocytes
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
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Memory cells
T-helper cells
• Increase functioning of B lymphocytes
T-suppressor cells
• Turn off functioning of B lymphocytes
T-cytotoxic cells
• Attack virally infected cells or tumor cells
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T Lymphocytes (cont.)
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Macrophages
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Active in phagocytosis of foreign material
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Produce cytokines called monokines
Help both B and T lymphocytes
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
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(pg. 84)
After phagocytosis, they process and present
antigen to lymphocytes
This stimulates lymphocytes to travel from
lymphoid tissue to the injury site
Amplify the immune response but do not
remember the antigen like lymphocytes
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Cytokines (Cont.)
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Cytokines (Cont.)
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
(pgs. 84-85) (Table 3-2)
Proteins made by cells that are able to
affect the behavior of other cells

Different cytokines have different functions.
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Major Divisions of the Immune
Response
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Humoral response
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(pgs. 85-86)
B lymphocytes are the primary cells.
Involves production of antibodies
Cell-mediated response

T lymphocytes are the primary cells.
• Lymphocytes may work alone or be assisted by
macrophages.

The cell-mediated portion regulates both major
responses.
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Major Divisions of the Immune
Response (cont.)
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Memory and Immunity
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(pg. 86)
The immune system has memory; the
inflammatory system does not.
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Some lymphocytes retain memory of an
antigen after an initial encounter.
This means the immune response will be faster
and stronger the next time an antigen enters
the body.
The retained memory is called immunity.
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Types of Immunity
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Passive Immunity
Active Immunity
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Passive Immunity
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
(pg. 86)
Using antibodies created by another
person to prevent infectious disease
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Natural
• When antibodies from the mother pass through the
placenta to the developing fetus

Acquired
• When antibodies are acquired through an injection
• Short lived but fast acting
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Active Immunity


(pg. 86)
Antibodies created by the person
themselves
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Natural
• Protection conferred following survival from an
infectious disease

Acquired
• Injection or ingestion of either altered pathogenic
microorganisms or products of those microorganisms
– immunization with a vaccine
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Immunopathology
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
(pg. 86)
The study of immune reactions involved in
disease
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The immune system can malfunction and
cause tissue damage.
• Hypersensitivity
• Autoimmune diseases
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Hypersensitivity (Cont.)
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(pgs. 87-88) (Table 3-3)
An allergic reaction
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An exaggerated response
Tissue destruction occurs as a result of the
immune response.
Four main types
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Hypersensitivity (Cont.)
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Type I Hypersensitivity
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(pg. 87)
Immediate (anaphylactic type)
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The reaction occurs within minutes after
exposure to an antigen.
Plasma cells produce IgE.
• IgE causes mast cells to release histamine, causing
increased dilation and permeability of blood vessels
and constricting smooth muscle in bronchioles of the
lungs.

The reaction may range from hay fever to
asthma and life-threatening anaphylaxis.
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Type II Hypersensitivity
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Cytotoxic type
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(pg. 87)
Antibody combines with an antigen bound to
the surface of tissue cells, usually a circulating
RBC (red blood cell).
Activated complement components, IgG
and IgM antibodies in blood, participate in
this type of hypersensitivity reaction.

This destroys the tissue that has the antigens
on the surface of its cells (e.g., Rh
incompatibility).
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Type III Hypersensitivity
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
(pg. 87)
Immune complex type (serum sickness)
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Immune complexes are formed between
microorganisms and antibody in circulating
blood.
• These complexes leave the blood and are deposited
in body tissues, where they cause an acute
inflammatory response.

Tissue destruction occurs following
phagocytosis by neutrophils.
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Type IV Hypersensitivity
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
(pg. 87)
Cell-mediated type (delayed)
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T lymphocytes that previously have been
introduced to an antigen cause damage to
tissue cells or recruit other cells.
Responsible for the rejection of tissue grafts
and transplanted organs
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Hypersensitivity to Drugs
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(pgs. 87-88)
Drugs can act as antigens.
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Topical administration may cause a greater
number of reactions than oral or parenteral
routes.
But the parenteral route may cause a more
widespread and severe reaction.
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Autoimmune Diseases
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Immunologic tolerance
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(pg. 88)
The body learns to determine self from nonself.
Autoimmune disorder
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The recognition mechanism breaks down;
some body cells are not tolerated and are
treated as foreign antigens.
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Immunodeficiency
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(pg. 88)
An immunopathologic condition
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A deficiency in number, function, or
interrelationships of the involved WBC’s and
their products
May be congenital or acquired
Infections and tumors may occur as a result of
the deficiency.
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Oral Diseases with Immunologic
Pathogenesis
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Aphthous ulcers
Urticaria and angioedema
Contact mucositis and dermatitis
Fixed drug eruptions
Erythema multiforme
Lichen planus
Reactive arthritis (Reiter syndrome)
Langerhans cell disease
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Aphthous Ulcers
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(pgs. 88-91)
Painful oral ulcers with an unclear cause
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Occur in about 20% of the population
Trauma is the most common precipitating
factor.
• May be caused by emotional stress or certain food
 May be associated with certain systemic
diseases
 Thought to have an immunologic pathogenesis
 Occur in three forms: minor, major, and
herpetiform
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Minor Aphthous Ulcers
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(pg. 89)
Discrete, round or oval ulcers
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Occur on movable mucosa
• Up to 1 cm in diameter with a erythematous halo
surrounding a yellowish-white fibrin surface


May have single or multiple lesions
May have a prodrome of 1 to 2 days
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Minor Aphthous Ulcers (cont.)
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Major Aphthous Ulcers
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(pgs. 89-90)
Larger (>1 cm), deeper, and longer lasting
than minor aphthous ulcers
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Very painful
Occur in the posterior of the mouth more often
than minor aphthous ulcers
May require several weeks to heal
May require a biopsy
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Major Aphthous Ulcers (cont.)
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Herpetiform Aphthous Ulcers
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(pgs. 89-90)
Tiny (1 to 2 mm)
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Resemble herpes simplex ulcers
Painful, generally occur in groups
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Diagnosis of Minor Aphthous
Ulcers (Cont.)
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Diagnosis of Minor Aphthous
Ulcers (Cont.)
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Clinical appearance
Location
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(pgs. 89-91) (Table 3-4)
Herpetic lesions appear on mucosa fixed to
bone, aphthous lesions appear on movable
tissue
Clinical history
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Aphthous ulcers do not produce systemic signs
or symptoms as do herpetic lesions
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Treatment of Minor Aphthous Ulcers
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(pg. 91)
Treatment
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
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There are several OTC medications such as
Orabase and Zilactin.
Topical or systemic steroids may help.
Topical anesthetic may help.
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Urticaria (Hives)
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Appears as multiple
areas of welldemarcated swelling of
skin
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(pg. 91)
May have itching (pruritis)
Lesions caused by
localized areas of
vascular permeability in
superficial connective
tissue
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Angioedema
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(pg. 91)
Lesions caused by
diffuse swelling due to
increased permeability
of deeper blood vessels


The skin covering the
swelling appears normal
Usually do not have
itching
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Urticaria and Angioedema
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(pgs. 91-92)
Often idiopathic cause
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
May be due to infection, trauma, emotional
stress, and certain systemic diseases
May be due to ingested allergens
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Contact Mucositis and Dermatitis
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Lesions resulting from contact of an
allergen with skin or mucosa
Involves CMI (cell-mediated immunity)
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
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(pg. 92)
The mucosa initially becomes erythematous
and edematous.
• Often there is burning and pruritus
Later, the area becomes white and scaly.
Treatment

Topical and/or systemic corticosteroids
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Contact Mucositis and Dermatitis
(cont.)
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Fixed Drug Eruptions
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Lesions that appear in the same site each
time a drug is introduced
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(pgs. 92-93)
Generally appear suddenly after a latent period
and subside when the drug is discontinued
May be single or multiple slightly raised,
reddish patches or clusters of macules on
the skin, or sometimes the mucous
membranes

May have pain or pruritis
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Fixed Drug Eruptions (cont.)
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A type of allergic reaction (Type III)
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
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Immune complexes are deposited along the
endothelial walls of blood vessels.
Inflammation causes vasculitis with damage to
the vessel wall.
This creates erythema and edema in
superficial layers of skin or mucosa.
Treatment

The drug causing the reaction should be
identified and discontinued.
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Erythema Multiforme
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
Cause is not clear; may be a
hypersensitivity reaction
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
(pgs. 93-94)
Most commonly occurs in young adults, affects
men more commonly than women
Target lesion

Characteristic skin lesion with concentric
erythematous rings alternating with normal skin
color
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Erythema Multiforme (cont.)
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Erythema Multiforme (cont.)
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Skin lesions can range from macules to
papules to bullae.
Oral lesions are usually ulcers

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

(pgs. 93-94)
Frequently form on lateral borders of the
tongue
Crusted and bleeding lips are frequently seen.
Gingival involvement is rare.
May be chronic or may have recurrent
acute episodes
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Erythema Multiforme (cont.)
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Erythema Multiforme (cont.)


(pgs. 93-94)
Stevens-Johnson
syndrome

The most severe form
• More extensive and
painful oral lesions
• Genital mucosa and
mucosa of eyes may be
involved.
• Lips generally are
encrusted and bloody.
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Erythema Multiforme (cont.)
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
Diagnosis


(pg. 93)
Based on clinical features and by exclusion of
other diseases
Treatment and prognosis


Topical or systemic corticosteroids
Eye lesions may lead to blindness.
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Lichen Planus
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
A benign, chronic disease affecting skin
and oral mucosa



Unknown cause
Lesions have characteristic Wickham striae
Most commonly on buccal mucosa


(pgs. 93-96)
Lesions may be on the tongue, lips, floor of
mouth, and gingiva.
Present in about 1% of the U.S. population


Most common in middle age
Slightly more common in women
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Lichen Planus (cont.)
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Types of Lichen Planus
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Reticular lichen planus
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Most common form
Erosive and bullous lichen planus
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
(pgs. 95-96)
Epithelium separates from connective tissue
Desquamative gingivitis can be caused by
lichen planus.
Skin lesions

2 to 4 mm papules most commonly in lumber
region, flexor surfaces of the wrist, anterior
ankle
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Types of Lichen Planus (cont.)
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Diagnosis of Lichen Planus


(pgs. 95-96)
Based on clinical
appearance and
possibly biopsy


Epithelial atypia
and dysplasia may
occur in lesions
that clinically
appear to be lichen
planus.
These lesions may
be premalignant.
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Treatment and Prognosis of Lichen
Planus


(pg. 96)
A chronic disease


Treated when symptomatic
Regular oral examination and biopsy of
suspicious lesions are necessary as these
patients may be at increased risk of
development of squamous cell carcinoma.
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Reactive Arthritis
(Reiter Syndrome)


Classic syndrome includes arthritis, urethritis, and
conjunctivitis, but all components may not be
present.




(pgs. 96-97)
An antigenic marker called HLA-B27 is present in most
patients, meaning there may be a genetic influence.
Probably an abnormal immune response to a microbial
antigen
Skin and mucous membrane lesions may be
observed.
May see aphthous ulcers, erythematous lesions,
and geographic tonguelike lesions
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Reactive Arthritis
(Reiter Syndrome) (cont.)
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Reactive Arthritis

Diagnosis



Clinical signs and symptoms
HLA-B27 antigenic marker
Treatment and prognosis


Disease lasts for weeks to months.
Recurrent attacks are common.
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Langerhans Cell Disease
(Histiocytosis X)


Includes three entities




(pgs. 97-98)
Letterer-Siwe disease
Hand-Schuller-Christian disease
Solitary eosinophilic granuloma
All have Langerhans cells and eosinophils.
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Langerhans Cell Disease
(Histiocytosis X) (cont.)
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Langerhans Cell Disease

A type of macrophage


An immunocompetent cell of the mononuclear
phagocyte series and participates in CMI
Treatment


Eosinophilic granuloma is treated by
conservative surgical excision.
Low-dose radiation may be used
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Autoimmune Diseases that Affect the
Oral Cavity






Sjögren Syndrome
Systemic Lupus Erythematosus
Pemphigus Vulgaris
Mucous Membrane Pemphigoid
Bullous Pemphigoid
Behçet Syndrome
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Autoimmune Diseases that Affect
the Oral Cavity (cont.)



(pgs. 98-105) (Table 3-5)
Several autoimmune diseases affect the oral
cavity.
The immune system treats the person’s own
cells and tissues as antigens.
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Autoimmune Diseases that Affect
the Oral Cavity (cont.)
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Sjögren Syndrome


(pgs. 99-100)
Affects the salivary and lacrimal glands


Results in a decrease in saliva and tears
causing a dry mouth (xerostomia) and dry eyes
(xerophthalmia)
The combination may be called sicca
syndrome.
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Sjögren Syndrome (cont.)


May be associated with other
autoimmune disorders




(pg. 99)
Primary Sjögren syndrome –
when it occurs alone
Secondary Sjögren syndrome when it occurs with other
autoimmune disorders
Patient may complain of oral
discomfort due to dry mouth.
May see loss of filiform and
fungiform papillae on the
dorsum of the tongue
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Sjögren Syndrome (cont.)


Affects both major and minor salivary
glands


(pg. 99)
Parotid gland enlargement occurs in about
50% of patients.
Biopsy reveals a characteristic
appearance.

Major salivary glands
• Replacement with lymphocytes and the presence of
islands of epithelium called epimyoepithelial islands

Minor salivary glands
• Aggregates of lymphocytes surrounding the salivary
gland ducts
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Sjögren Syndrome (cont.)
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Sjögren Syndrome (cont.)

Patient may complain of burning and
itching of eyes and photophobia.


Severe eye involvement may lead to ulceration
and opacification of the eyes.
Raynaud phenomenon

20% of these patients will have this disorder
affecting fingers and toes
• Initial pallor and subsequent cyanosis of skin due to
cold or stress
• Hyperemia when blood vessels are warmed
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Sjögren Syndrome (cont.)

90% of these patients have a positive
response to rheumatoid factor, an antibody
to IgG present in serum


It is an antibody to an antibody.
Other autoantibodies, anti-Sjögren
syndrome A, and anti-Sjögren syndrome B
are also present.
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Diagnosis and Management of
Sjögren Syndrome


Diagnosis is made when two of three
components are present.




(pg. 100)
Xerostomia
• Measurement of salivary flow and biopsy can help
Keratoconjunctivitis sicca
• Confirmed with eye examination
Rheumatoid arthritis
For most patients, the course of the
disease is chronic and benign but these
patients are at risk for the development of
other more serious diseases.
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Diagnosis and Management of
Sjögren Syndrome (cont.)
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Treatment of Sjögren Syndrome


(pg. 100)
Symptomatic

Nonsteroidal antiinflammatory agents for
arthritis
• May need corticosteroids and immunosuppressive
drugs for severe cases


Saliva substitutes for xerostomia
• Humidifier, sugarless gum, or lozenges
• Pilocarpine
Glasses and/or artificial tears to protect eyes
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Systemic Lupus Erythematosus
(SLE)


An acute and chronic inflammatory
autoimmune disease


(pgs. 100-102)
No known cause
Affects women 8 times more frequently
than men, predominantly during
childbearing years

Three times more frequent in black women
than in white women
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Systemic Lupus Erythematosus
(SLE) (cont.)

A syndrome with a wide range of disease
activity



Usually chronic and progressive
• Periods of remission and exacerbation
Autoantibodies to DNA are present in
serum
May have a genetic component
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Clinical Features of Systemic
Lupus Erythematosus (SLE)


Skin lesions occur in 85% of individuals




(pgs. 101-102)
“Butterfly” rash on bridge of nose
May be erythematous lesions on fingertips
Arthritis and arthralgia are common.
Oral lesions accompany skin lesions in
about 25% of patients with discoid LE.

Erythematous plaques or erosions
• May have white striae; resemble lichen planus but
are less symmetric
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Clinical Features of Systemic Lupus
Erythematosus (SLE) (cont.)
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Diagnosis of Systemic Lupus
Erythematosus (SLE)


(pgs. 101-102)
Usually based on multiorgan involvement
and presence of antinuclear antibodies in
serum

Inflammatory infiltrate is around blood vessels
in connective tissue.
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Treatment and Prognosis of Systemic
Lupus Erythematosus (SLE)

Aspirin and antiinflammatory drugs for mild
signs and symptoms


Hydroxychloroquine and corticosteroids along
with immunosuppressive agents may be used.
The text recommends consultation with the
patient’s physician before initiating dental
treatment.
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Pemphigus Vulgaris


A severe, progressive autoimmune
disease affecting skin and mucous
membranes


(pgs. 102-104)
Characterized by intraepithelial blister
formation resulting from acantholysis, a
breakdown of cellular adhesion between
epithelial cells
Genetic and ethnic factors have been
reported.

Often seen in Ashkenazic Jews
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Pemphigus Vulgaris (cont.)


(pg. 103)
Oral lesions


The first signs of disease occur in the oral
cavity in more than 50% of cases.
• May be shallow ulcers, to fragile vesicles, to bullae
Nikolsky sign
• Rubbing with a finger can produce a bullae.
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Pemphigus Vulgaris (cont.)
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Pemphigus Vulgaris (cont.)


Skin lesions


(pgs. 103-104)
Erythema, bullae,
erosions, ulcers
Microscopic
appearance

Acantholytic cells
• The loss of
attachment
between epithelial
cells leads to cells
that appear
rounded.

Tzanck cells
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Diagnosis of Pemphigus Vulgaris

Made by biopsy and microscopic
examination


Direct immunofluorescence
• Identifies autoantibodies present in tissue
Indirect immunofluorecence
• The patient’s serum is used to detect circulating
autoantibodies.
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Treatment and Prognosis of
Pemphigus Vulgaris

High doses of corticosteroids


May include immunosuppressive drugs
Mortality rate of 8% to 10% in 5 years is related
to complications of corticosteroid treatment.
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Mucous Membrane Pemphigoid
(Cicatricial Pemphigoid) (BMMP)


A chronic autoimmune disease


Affects oral mucosa, conjunctiva, genital
mucosa, and skin
Not as severe as pemphigus vulgaris


(pgs. 104-105)
Gingival lesions have been called
desquamative gingivitis, but this may be seen
with lichen planus and pemphigus as well.
Will see positive Nikolsky sign
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Mucous Membrane Pemphigoid (Cicatricial
Pemphigoid) (BMMP) (cont.)
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Diagnosis of Mucous Membrane
Pemphigoid


(pg. 104)
Made by biopsy and
histologic
examination


No degeneration of
epithelium occurs
An inflammatory
infiltrate, usually with
predominant plasma
cells and eosinophils,
is seen in connective
tissue.
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Treatment and Prognosis of
Mucous Membrane Pemphigoid

A chronic disease with a benign course



Topical corticosteroid for mild cases
Systemic corticosteroids may be required for
more severe cases.
Eye lesions can lead to eye damage.
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Bullous Pemphigoid


Some investigators believe bullous and
mucous membrane pemphigoid are
variants of a single disease, but 80% of
these patients are older than 60.


(pg. 105)
Oral lesions are less common than in cicatricial
pemphigoid.
Treatment

Systemic corticosteroids and nonsteroidal
antiinflammatory drugs
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Behçet Syndrome


A chronic, recurrent autoimmune disease




(pg. 105)
Primarily oral ulcers, genital ulcers, ocular
inflammation
No sex predilection; mean onset is 30 years
Autoantibodies to human mucosa may be
found.
Oral ulcers are similar in appearance to
aphthous ulcers.
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Behçet Syndrome (cont.)
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Diagnosis of Behçet Syndrome

Requires that two of three types of lesions
(oral, genital, and ocular) be present.

A pustular lesion after needle puncture
suggests Behçet syndrome.
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Treatment and Prognosis of
Behçet Syndrome


(pg. 105)
Systemic and topical corticosteroids

Chlorambucil is used for ocular lesions.
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Discussion Questions







What is an autoimmune disorder?
What is the difference between an antigen and
an antibody?
What are the differences between active and
passive immunity?
What oral diseases have an immunologic
pathogenesis?
What is an autoimmune disorder?
What are the oral symptoms of Sjögren
syndrome?
What are differences between pemphigus and
pemphigoid?
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