1 elsevier items and derived items © 2009 by saunders, an imprint of elsevier inc. chapter 3...

1Elsevier items and derived items © 2009 by Saunders, an imprint of Elsevier Inc.

Chapter 3

Immunity


Outline Outline The Acquired Immune ResponseThe Acquired Immune Response AntigensAntigens Cellular Involvement in the Immune ResponseCellular Involvement in the Immune Response Major Divisions of the Immune ResponseMajor Divisions of the Immune Response Memory and ImmunityMemory and Immunity Types of ImmunityTypes of Immunity ImmunopathologyImmunopathology Oral Diseases with Immunologic PathogenesisOral Diseases with Immunologic Pathogenesis Autoimmune Diseases that Affect the Oral Autoimmune Diseases that Affect the Oral

CavityCavity


The Acquired Immune ResponseThe Acquired Immune Response

(pg. 82)(pg. 82) Defends the body against injuryDefends the body against injury

Has the capacity to remember and respond Has the capacity to remember and respond more quickly the second time a foreign material more quickly the second time a foreign material enters the bodyenters the body

Works with the inflammatory response and a Works with the inflammatory response and a working repair processworking repair process

Involves white blood cells, especially Involves white blood cells, especially lymphocytes and their productslymphocytes and their products


AntigensAntigens

(pg. 82)(pg. 82) Foreign substancesForeign substances

Mainly proteins, often microorganisms and Mainly proteins, often microorganisms and their toxinstheir toxins

Human cells that have been transformedHuman cells that have been transformed May be tumor cells, or cells infected with May be tumor cells, or cells infected with

virusesviruses


Antigens (cont.)Antigens (cont.)

Human tissueHuman tissue Organ transplants, tissue grafts, incompatible Organ transplants, tissue grafts, incompatible

blood types during a transfusionblood types during a transfusion Autoimmune diseasesAutoimmune diseases

Tissue from the person’s own body becomes Tissue from the person’s own body becomes an antigenan antigen


Cellular Involvement in the Cellular Involvement in the Immune ResponseImmune Response

B lymphocytesB lymphocytes T lymphocytesT lymphocytes MacrophagesMacrophages CytokinesCytokines


Cellular Involvement in the Cellular Involvement in the Immune Response (cont.) Immune Response (cont.)

(pg. 83)(pg. 83) Cells that are able to recognize and Cells that are able to recognize and

respond to antigenrespond to antigen Derived from precursor cells in bone marrow Derived from precursor cells in bone marrow

(stem cells)(stem cells) 20% to 25% of the WBC (white blood cell) 20% to 25% of the WBC (white blood cell)

populationpopulation Two main typesTwo main types

B lymphocytes (B cells)B lymphocytes (B cells) T lymphocytes (T cells)T lymphocytes (T cells)

Also there are natural killer cells (NK cells)Also there are natural killer cells (NK cells) Can destroy cells recognized as foreign without Can destroy cells recognized as foreign without

recognizing specific antigensrecognizing specific antigens


Cellular Involvement in the Cellular Involvement in the Immune Response (cont.)Immune Response (cont.)


B LymphocytesB Lymphocytes

(pgs. 83-84)(pgs. 83-84) Mature and reside in lymphoid tissueMature and reside in lymphoid tissue

Lymph nodes, tonsils, and other body tissueLymph nodes, tonsils, and other body tissue B lymphocytes travel to the site of injury B lymphocytes travel to the site of injury

when stimulated by antigenwhen stimulated by antigen Two main typesTwo main types

• Plasma cells Plasma cells Produce specific antibodiesProduce specific antibodies

• B memory cellB memory cell Retains the memory of previously encountered antigen Retains the memory of previously encountered antigen

and will clone itself in the presence of antigenand will clone itself in the presence of antigen


B Lymphocytes (cont.)B Lymphocytes (cont.)


Plasma CellsPlasma Cells

Produce antibodies that are categorized Produce antibodies that are categorized into 5 classes of immunoglobulins, which into 5 classes of immunoglobulins, which are carried in blood serumare carried in blood serum All have the same basic “Y” structure, but have All have the same basic “Y” structure, but have

an area with variable (V) structure at the tips of an area with variable (V) structure at the tips of the Ythe Y

The stem of the Y is constant (C) for all 5 The stem of the Y is constant (C) for all 5 types, and links the antibody to other types, and links the antibody to other components of the immune responsecomponents of the immune response

Immune complexImmune complex Antigen combined with antibodyAntigen combined with antibody


T LymphocytesT Lymphocytes

(pgs. 83, 85)(pgs. 83, 85) Travel to the thymus and matureTravel to the thymus and mature

The thymus is large in an infant, shrinks as the The thymus is large in an infant, shrinks as the child matureschild matures

Several different types of T lymphocytesSeveral different types of T lymphocytes Memory cellsMemory cells T-helper cellsT-helper cells

• Increase functioning of B lymphocytesIncrease functioning of B lymphocytes T-suppressor cellsT-suppressor cells

• Turn off functioning of B lymphocytesTurn off functioning of B lymphocytes T-cytotoxic cellsT-cytotoxic cells

• Attack virally infected cells or tumor cellsAttack virally infected cells or tumor cells


T Lymphocytes (cont.)T Lymphocytes (cont.)


MacrophagesMacrophages

(pg. 84)(pg. 84) Active in phagocytosis of foreign materialActive in phagocytosis of foreign material

Produce cytokines called monokinesProduce cytokines called monokines Help both B and T lymphocytesHelp both B and T lymphocytes

After phagocytosis, they process and present After phagocytosis, they process and present antigen to lymphocytesantigen to lymphocytes

This stimulates lymphocytes to travel from This stimulates lymphocytes to travel from lymphoid tissue to the injury sitelymphoid tissue to the injury site

Amplify the immune response but do not Amplify the immune response but do not remember the antigen like lymphocytesremember the antigen like lymphocytes


Cytokines (Cont.)Cytokines (Cont.)


Cytokines (Cont.)Cytokines (Cont.)

(pgs. 84-85) (Table 3-2)(pgs. 84-85) (Table 3-2) Proteins made by cells that are able to Proteins made by cells that are able to

affect the behavior of other cellsaffect the behavior of other cells Different cytokines have different functions.Different cytokines have different functions.


Major Divisions of the Immune Major Divisions of the Immune ResponseResponse

(pgs. 85-86)(pgs. 85-86) Humoral responseHumoral response

B lymphocytes are the primary cells.B lymphocytes are the primary cells. Involves production of antibodiesInvolves production of antibodies

Cell-mediated responseCell-mediated response T lymphocytes are the primary cells.T lymphocytes are the primary cells.

• Lymphocytes may work alone or be assisted by Lymphocytes may work alone or be assisted by macrophages.macrophages.

The cell-mediated portion regulates both major The cell-mediated portion regulates both major responses.responses.


Major Divisions of the Immune Major Divisions of the Immune Response (cont.)Response (cont.)


Memory and ImmunityMemory and Immunity

(pg. 86)(pg. 86) The immune system has memory; the The immune system has memory; the

inflammatory system does not.inflammatory system does not. Some lymphocytes retain memory of an Some lymphocytes retain memory of an

antigen after an initial encounter.antigen after an initial encounter. This means the immune response will be faster This means the immune response will be faster

and stronger the next time an antigen enters and stronger the next time an antigen enters the body.the body.

The retained memory is called immunity.The retained memory is called immunity.


Types of ImmunityTypes of Immunity

Passive ImmunityPassive Immunity Active ImmunityActive Immunity


Passive ImmunityPassive Immunity

(pg. 86)(pg. 86) Using antibodies created by another Using antibodies created by another

person to prevent infectious diseaseperson to prevent infectious disease NaturalNatural

• When antibodies from the mother pass through the When antibodies from the mother pass through the placenta to the developing fetusplacenta to the developing fetus

AcquiredAcquired• When antibodies are acquired through an injectionWhen antibodies are acquired through an injection• Short lived but fast actingShort lived but fast acting


Active ImmunityActive Immunity

(pg. 86)(pg. 86) Antibodies created by the person Antibodies created by the person

themselvesthemselves NaturalNatural

• Protection conferred following survival from an Protection conferred following survival from an infectious diseaseinfectious disease

AcquiredAcquired• Injection or ingestion of either altered pathogenic Injection or ingestion of either altered pathogenic

microorganisms or products of those microorganisms or products of those microorganisms – immunization with a vaccinemicroorganisms – immunization with a vaccine


ImmunopathologyImmunopathology

(pg. 86)(pg. 86) The study of immune reactions involved in The study of immune reactions involved in

diseasedisease The immune system can malfunction and The immune system can malfunction and

cause tissue damage.cause tissue damage.• HypersensitivityHypersensitivity• Autoimmune diseasesAutoimmune diseases


Hypersensitivity (Cont.)Hypersensitivity (Cont.)

(pgs. 87-88) (Table 3-3)(pgs. 87-88) (Table 3-3) An allergic reactionAn allergic reaction

An exaggerated responseAn exaggerated response Tissue destruction occurs as a result of the Tissue destruction occurs as a result of the

immune response.immune response. Four main typesFour main types


Hypersensitivity (Cont.)Hypersensitivity (Cont.)


Type I HypersensitivityType I Hypersensitivity

(pg. 87)(pg. 87) Immediate (anaphylactic type)Immediate (anaphylactic type)

The reaction occurs within minutes after The reaction occurs within minutes after exposure to an antigen.exposure to an antigen.

Plasma cells produce IgE.Plasma cells produce IgE.• IgE causes mast cells to release histamine, causing IgE causes mast cells to release histamine, causing

increased dilation and permeability of blood vessels increased dilation and permeability of blood vessels and constricting smooth muscle in bronchioles of the and constricting smooth muscle in bronchioles of the lungs.lungs.

The reaction may range from hay fever to The reaction may range from hay fever to asthma and life-threatening anaphylaxis.asthma and life-threatening anaphylaxis.


Type II HypersensitivityType II Hypersensitivity

(pg. 87)(pg. 87) Cytotoxic typeCytotoxic type

Antibody combines with an antigen bound to Antibody combines with an antigen bound to the surface of tissue cells, usually a circulating the surface of tissue cells, usually a circulating RBC (red blood cell).RBC (red blood cell).

Activated complement components, IgG Activated complement components, IgG and IgM antibodies in blood, participate in and IgM antibodies in blood, participate in this type of hypersensitivity reaction.this type of hypersensitivity reaction. This destroys the tissue that has the antigens This destroys the tissue that has the antigens

on the surface of its cells (e.g., Rh on the surface of its cells (e.g., Rh incompatibility).incompatibility).


Type III HypersensitivityType III Hypersensitivity

(pg. 87)(pg. 87) Immune complex type (serum sickness)Immune complex type (serum sickness)

Immune complexes are formed between Immune complexes are formed between microorganisms and antibody in circulating microorganisms and antibody in circulating blood.blood.• These complexes leave the blood and are deposited These complexes leave the blood and are deposited

in body tissues, where they cause an acute in body tissues, where they cause an acute inflammatory response.inflammatory response.

Tissue destruction occurs following Tissue destruction occurs following phagocytosis by neutrophils.phagocytosis by neutrophils.


Type IV HypersensitivityType IV Hypersensitivity

(pg. 87)(pg. 87) Cell-mediated type (delayed)Cell-mediated type (delayed)

T lymphocytes that previously have been T lymphocytes that previously have been introduced to an antigen cause damage to introduced to an antigen cause damage to tissue cells or recruit other cells.tissue cells or recruit other cells.

Responsible for the rejection of tissue grafts Responsible for the rejection of tissue grafts and transplanted organsand transplanted organs


Hypersensitivity to DrugsHypersensitivity to Drugs

(pgs. 87-88)(pgs. 87-88) Drugs can act as antigens.Drugs can act as antigens.

Topical administration may cause a greater Topical administration may cause a greater number of reactions than oral or parenteral number of reactions than oral or parenteral routes.routes.

But the parenteral route may cause a more But the parenteral route may cause a more widespread and severe reaction.widespread and severe reaction.


Autoimmune DiseasesAutoimmune Diseases

(pg. 88)(pg. 88) Immunologic toleranceImmunologic tolerance

The body learns to determine self from nonself.The body learns to determine self from nonself. Autoimmune disorderAutoimmune disorder

The recognition mechanism breaks down; The recognition mechanism breaks down; some body cells are not tolerated and are some body cells are not tolerated and are treated as foreign antigens.treated as foreign antigens.


ImmunodeficiencyImmunodeficiency

(pg. 88)(pg. 88) An immunopathologic conditionAn immunopathologic condition

A deficiency in number, function, or A deficiency in number, function, or interrelationships of the involved WBC’s and interrelationships of the involved WBC’s and their productstheir products

May be congenital or acquiredMay be congenital or acquired Infections and tumors may occur as a result of Infections and tumors may occur as a result of

the deficiency.the deficiency.


Oral Diseases with Immunologic Oral Diseases with Immunologic PathogenesisPathogenesis

Aphthous ulcersAphthous ulcers Urticaria and angioedemaUrticaria and angioedema Contact mucositis and dermatitisContact mucositis and dermatitis Fixed drug eruptionsFixed drug eruptions Erythema multiformeErythema multiforme Lichen planusLichen planus Reactive arthritis (Reiter syndrome)Reactive arthritis (Reiter syndrome) Langerhans cell disease Langerhans cell disease


Aphthous UlcersAphthous Ulcers

(pgs. 88-91)(pgs. 88-91) Painful oral ulcers with an unclear causePainful oral ulcers with an unclear cause

Occur in about 20% of the populationOccur in about 20% of the population Trauma is the most common precipitating Trauma is the most common precipitating

factor.factor.• May be caused by emotional stress or certain foodMay be caused by emotional stress or certain food

May be associated with certain systemic May be associated with certain systemic diseasesdiseases

Thought to have an immunologic pathogenesisThought to have an immunologic pathogenesis Occur in three forms: minor, major, and Occur in three forms: minor, major, and

herpetiformherpetiform


Minor Aphthous UlcersMinor Aphthous Ulcers

(pg. 89)(pg. 89) Discrete, round or oval ulcers Discrete, round or oval ulcers

Occur on movable mucosaOccur on movable mucosa• Up to 1 cm in diameter with a erythematous halo Up to 1 cm in diameter with a erythematous halo

surrounding a yellowish-white fibrin surfacesurrounding a yellowish-white fibrin surface May have single or multiple lesionsMay have single or multiple lesions May have a prodrome of 1 to 2 daysMay have a prodrome of 1 to 2 days


Minor Aphthous Ulcers (cont.)Minor Aphthous Ulcers (cont.)


Major Aphthous UlcersMajor Aphthous Ulcers

(pgs. 89-90)(pgs. 89-90) Larger (>1 cm), deeper, and longer lasting Larger (>1 cm), deeper, and longer lasting

than minor aphthous ulcersthan minor aphthous ulcers Very painfulVery painful Occur in the posterior of the mouth more often Occur in the posterior of the mouth more often

than minor aphthous ulcersthan minor aphthous ulcers May require several weeks to healMay require several weeks to heal May require a biopsyMay require a biopsy


Major Aphthous Ulcers (cont.)Major Aphthous Ulcers (cont.)


Herpetiform Aphthous UlcersHerpetiform Aphthous Ulcers

(pgs. 89-90)(pgs. 89-90) Tiny (1 to 2 mm)Tiny (1 to 2 mm)

Resemble herpes simplex ulcersResemble herpes simplex ulcers Painful, generally occur in groupsPainful, generally occur in groups


Diagnosis of Minor Aphthous Diagnosis of Minor Aphthous Ulcers (Cont.)Ulcers (Cont.)


Diagnosis of Minor Aphthous Diagnosis of Minor Aphthous Ulcers (Cont.)Ulcers (Cont.)

(pgs. 89-91) (Table 3-4)(pgs. 89-91) (Table 3-4) Clinical appearanceClinical appearance LocationLocation

Herpetic lesions appear on mucosa fixed to Herpetic lesions appear on mucosa fixed to bone, aphthous lesions appear on movable bone, aphthous lesions appear on movable tissuetissue

Clinical historyClinical history Aphthous ulcers do not produce systemic signs Aphthous ulcers do not produce systemic signs

or symptoms as do herpetic lesions or symptoms as do herpetic lesions


Treatment of Minor Aphthous UlcersTreatment of Minor Aphthous Ulcers

(pg. 91)(pg. 91) TreatmentTreatment

There are several OTC medications such as There are several OTC medications such as Orabase and Zilactin.Orabase and Zilactin.

Topical or systemic steroids may help.Topical or systemic steroids may help. Topical anesthetic may help.Topical anesthetic may help.


Urticaria (Hives)Urticaria (Hives)

(pg. 91)(pg. 91) Appears as multiple Appears as multiple

areas of well-areas of well-demarcated swelling of demarcated swelling of skinskin May have itching (pruritis)May have itching (pruritis)

Lesions caused by Lesions caused by localized areas of localized areas of vascular permeability in vascular permeability in superficial connective superficial connective tissuetissue


AngioedemaAngioedema

(pg. 91)(pg. 91) Lesions caused by Lesions caused by

diffuse swelling due to diffuse swelling due to increased permeability increased permeability of deeper blood vesselsof deeper blood vessels The skin covering the The skin covering the

swelling appears normalswelling appears normal Usually do not have Usually do not have

itchingitching


Urticaria and AngioedemaUrticaria and Angioedema

(pgs. 91-92)(pgs. 91-92) Often idiopathic causeOften idiopathic cause

May be due to infection, trauma, emotional May be due to infection, trauma, emotional stress, and certain systemic diseasesstress, and certain systemic diseases

May be due to ingested allergensMay be due to ingested allergens


Contact Mucositis and DermatitisContact Mucositis and Dermatitis

(pg. 92)(pg. 92) Lesions resulting from contact of an Lesions resulting from contact of an

allergen with skin or mucosaallergen with skin or mucosa Involves CMI (cell-mediated immunity)Involves CMI (cell-mediated immunity)

The mucosa initially becomes erythematous The mucosa initially becomes erythematous and edematous.and edematous.• Often there is burning and pruritusOften there is burning and pruritus

Later, the area becomes white and scaly.Later, the area becomes white and scaly. TreatmentTreatment

Topical and/or systemic corticosteroidsTopical and/or systemic corticosteroids


Contact Mucositis and Dermatitis Contact Mucositis and Dermatitis (cont.)(cont.)


Fixed Drug EruptionsFixed Drug Eruptions

(pgs. 92-93)(pgs. 92-93) Lesions that appear in the same site each Lesions that appear in the same site each

time a drug is introducedtime a drug is introduced Generally appear suddenly after a latent period Generally appear suddenly after a latent period

and subside when the drug is discontinuedand subside when the drug is discontinued May be single or multiple slightly raised, May be single or multiple slightly raised,

reddish patches or clusters of macules on reddish patches or clusters of macules on the skin, or sometimes the mucous the skin, or sometimes the mucous membranesmembranes May have pain or pruritisMay have pain or pruritis


Fixed Drug Eruptions (cont.)Fixed Drug Eruptions (cont.)

A type of allergic reaction (Type III)A type of allergic reaction (Type III) Immune complexes are deposited along the Immune complexes are deposited along the

endothelial walls of blood vessels.endothelial walls of blood vessels. Inflammation causes vasculitis with damage to Inflammation causes vasculitis with damage to

the vessel wall.the vessel wall. This creates erythema and edema in This creates erythema and edema in

superficial layers of skin or mucosa.superficial layers of skin or mucosa. TreatmentTreatment

The drug causing the reaction should be The drug causing the reaction should be identified and discontinued.identified and discontinued.


Erythema MultiformeErythema Multiforme

(pgs. 93-94) (pgs. 93-94) Cause is not clear; may be a Cause is not clear; may be a

hypersensitivity reactionhypersensitivity reaction Most commonly occurs in young adults, affects Most commonly occurs in young adults, affects

men more commonly than womenmen more commonly than women Target lesionTarget lesion

Characteristic skin lesion with concentric Characteristic skin lesion with concentric erythematous rings alternating with normal skin erythematous rings alternating with normal skin colorcolor


Erythema Multiforme (cont.)Erythema Multiforme (cont.)



(pgs. 93-94)(pgs. 93-94) Skin lesions can range from macules to Skin lesions can range from macules to

papules to bullae.papules to bullae. Oral lesions are usually ulcersOral lesions are usually ulcers

Frequently form on lateral borders of the Frequently form on lateral borders of the tonguetongue

Crusted and bleeding lips are frequently seen.Crusted and bleeding lips are frequently seen. Gingival involvement is rare.Gingival involvement is rare.

May be chronic or may have recurrent May be chronic or may have recurrent acute episodesacute episodes



(pgs. 93-94)(pgs. 93-94) Stevens-Johnson Stevens-Johnson

syndromesyndrome The most severe formThe most severe form

• More extensive and More extensive and painful oral lesionspainful oral lesions

• Genital mucosa and Genital mucosa and mucosa of eyes may be mucosa of eyes may be involved.involved.

• Lips generally are Lips generally are encrusted and bloody.encrusted and bloody.



(pg. 93)(pg. 93) DiagnosisDiagnosis

Based on clinical features and by exclusion of Based on clinical features and by exclusion of other diseasesother diseases

Treatment and prognosisTreatment and prognosis Topical or systemic corticosteroidsTopical or systemic corticosteroids Eye lesions may lead to blindness.Eye lesions may lead to blindness.


Lichen PlanusLichen Planus

(pgs. 93-96)(pgs. 93-96) A benign, chronic disease affecting skin A benign, chronic disease affecting skin

and oral mucosaand oral mucosa Unknown causeUnknown cause Lesions have characteristic Wickham striaeLesions have characteristic Wickham striae

Most commonly on buccal mucosaMost commonly on buccal mucosa Lesions may be on the tongue, lips, floor of Lesions may be on the tongue, lips, floor of

mouth, and gingiva.mouth, and gingiva. Present in about 1% of the U.S. populationPresent in about 1% of the U.S. population

Most common in middle ageMost common in middle age Slightly more common in womenSlightly more common in women


Lichen Planus (cont.)Lichen Planus (cont.)


Types of Lichen PlanusTypes of Lichen Planus

(pgs. 95-96) (pgs. 95-96) Reticular lichen planusReticular lichen planus

Most common formMost common form Erosive and bullous lichen planusErosive and bullous lichen planus

Epithelium separates from connective tissueEpithelium separates from connective tissue Desquamative gingivitis can be caused by Desquamative gingivitis can be caused by

lichen planus.lichen planus. Skin lesionsSkin lesions

2 to 4 mm papules most commonly in lumber 2 to 4 mm papules most commonly in lumber region, flexor surfaces of the wrist, anterior region, flexor surfaces of the wrist, anterior ankleankle


Types of Lichen Planus (cont.)Types of Lichen Planus (cont.)


Diagnosis of Lichen PlanusDiagnosis of Lichen Planus

(pgs. 95-96)(pgs. 95-96) Based on clinical Based on clinical

appearance and appearance and possibly biopsypossibly biopsy Epithelial atypia Epithelial atypia

and dysplasia may and dysplasia may occur in lesions occur in lesions that clinically that clinically appear to be lichen appear to be lichen planus.planus.

These lesions may These lesions may

be premalignantbe premalignant..


Treatment and Prognosis of Lichen Treatment and Prognosis of Lichen PlanusPlanus

(pg. 96)(pg. 96) A chronic diseaseA chronic disease

Treated when symptomaticTreated when symptomatic Regular oral examination and biopsy of Regular oral examination and biopsy of

suspicious lesions are necessary as these suspicious lesions are necessary as these patients may be at increased risk of patients may be at increased risk of development of squamous cell carcinoma.development of squamous cell carcinoma.


Reactive Arthritis Reactive Arthritis (Reiter Syndrome) (Reiter Syndrome)

(pgs. 96-97)(pgs. 96-97) Classic syndrome includes arthritis, urethritis, and Classic syndrome includes arthritis, urethritis, and

conjunctivitis, but all components may not be conjunctivitis, but all components may not be present.present. An antigenic marker called HLA-B27 is present in most An antigenic marker called HLA-B27 is present in most

patients, meaning there may be a genetic influence.patients, meaning there may be a genetic influence. Probably an abnormal immune response to a microbial Probably an abnormal immune response to a microbial

antigenantigen Skin and mucous membrane lesions may be Skin and mucous membrane lesions may be

observed.observed. May see aphthous ulcers, erythematous lesions, May see aphthous ulcers, erythematous lesions,

and geographic tonguelike lesionsand geographic tonguelike lesions


Reactive Arthritis Reactive Arthritis (Reiter Syndrome) (cont.) (Reiter Syndrome) (cont.)


Reactive Arthritis Reactive Arthritis

DiagnosisDiagnosis Clinical signs and symptomsClinical signs and symptoms HLA-B27 antigenic markerHLA-B27 antigenic marker

Treatment and prognosisTreatment and prognosis Disease lasts for weeks to months.Disease lasts for weeks to months. Recurrent attacks areRecurrent attacks are common. common.


Langerhans Cell Disease Langerhans Cell Disease (Histiocytosis X)(Histiocytosis X)

(pgs. 97-98)(pgs. 97-98) Includes three entitiesIncludes three entities

Letterer-Siwe diseaseLetterer-Siwe disease Hand-Schuller-Christian diseaseHand-Schuller-Christian disease Solitary eosinophilic granuloma Solitary eosinophilic granuloma

All have Langerhans cells and eosinophils.All have Langerhans cells and eosinophils.


Langerhans Cell Disease Langerhans Cell Disease (Histiocytosis X) (cont.)(Histiocytosis X) (cont.)


Langerhans Cell DiseaseLangerhans Cell Disease

A type of macrophageA type of macrophage An immunocompetent cell of the mononuclear An immunocompetent cell of the mononuclear

phagocyte series and participates in CMIphagocyte series and participates in CMI TreatmentTreatment

Eosinophilic granuloma is treated by Eosinophilic granuloma is treated by conservative surgical excision.conservative surgical excision.

Low-dose radiation may be usedLow-dose radiation may be used


Autoimmune Diseases that Affect the Autoimmune Diseases that Affect the Oral CavityOral Cavity

Sjögren SyndromeSjögren Syndrome Systemic Lupus ErythematosusSystemic Lupus Erythematosus Pemphigus VulgarisPemphigus Vulgaris Mucous Membrane PemphigoidMucous Membrane Pemphigoid Bullous PemphigoidBullous Pemphigoid Behçet SyndromeBehçet Syndrome


Autoimmune Diseases that Affect Autoimmune Diseases that Affect the Oral Cavity (cont.)the Oral Cavity (cont.)

(pgs. 98-105) (Table 3-5)(pgs. 98-105) (Table 3-5) Several autoimmune diseases affect the oral Several autoimmune diseases affect the oral

cavity.cavity. The immune system treats the person’s own The immune system treats the person’s own

cells and tissues as antigens.cells and tissues as antigens.


Autoimmune Diseases that Affect Autoimmune Diseases that Affect the Oral Cavity (cont.)the Oral Cavity (cont.)


Sjögren SyndromeSjögren Syndrome

(pgs. 99-100)(pgs. 99-100) Affects the salivary and lacrimal glandsAffects the salivary and lacrimal glands

Results in a decrease in saliva and tears Results in a decrease in saliva and tears causing a dry mouth (xerostomia) and dry eyes causing a dry mouth (xerostomia) and dry eyes (xerophthalmia)(xerophthalmia)

The combination may be called sicca The combination may be called sicca syndrome.syndrome.


Sjögren Syndrome (cont.)Sjögren Syndrome (cont.)

(pg. 99)(pg. 99) May be associated with other May be associated with other

autoimmune disordersautoimmune disorders Primary Sjögren syndrome – Primary Sjögren syndrome –

when it occurs alonewhen it occurs alone Secondary Sjögren syndrome - Secondary Sjögren syndrome -

when it occurs with other when it occurs with other autoimmune disordersautoimmune disorders

Patient may complain of oral Patient may complain of oral discomfort due to dry mouth.discomfort due to dry mouth.

May see loss of filiform and May see loss of filiform and fungiform papillae on the fungiform papillae on the dorsum of the tonguedorsum of the tongue



(pg. 99)(pg. 99) Affects both major and minor salivary Affects both major and minor salivary

glandsglands Parotid gland enlargement occurs in about Parotid gland enlargement occurs in about

50% of patients.50% of patients. Biopsy reveals a characteristic Biopsy reveals a characteristic

appearance.appearance. Major salivary glandsMajor salivary glands

• Replacement with lymphocytes and the presence of Replacement with lymphocytes and the presence of islands of epithelium called epimyoepithelial islandsislands of epithelium called epimyoepithelial islands

Minor salivary glands Minor salivary glands • Aggregates of lymphocytes surrounding the salivary Aggregates of lymphocytes surrounding the salivary

gland ductsgland ducts



Patient may complain of burning and Patient may complain of burning and itching of eyes and photophobia.itching of eyes and photophobia. Severe eye involvement may lead to ulceration Severe eye involvement may lead to ulceration

and opacification of the eyes.and opacification of the eyes. Raynaud phenomenonRaynaud phenomenon

20% of these patients will have this disorder 20% of these patients will have this disorder affecting fingers and toesaffecting fingers and toes• Initial pallor and subsequent cyanosis of skin due to Initial pallor and subsequent cyanosis of skin due to

cold or stresscold or stress• Hyperemia when blood vessels are warmedHyperemia when blood vessels are warmed



90% of these patients have a positive 90% of these patients have a positive response to rheumatoid factor, an antibody response to rheumatoid factor, an antibody to IgG present in serumto IgG present in serum It is an antibody to an antibody.It is an antibody to an antibody.

Other autoantibodies, anti-Sjögren Other autoantibodies, anti-Sjögren syndrome A, and anti-Sjögren syndrome B syndrome A, and anti-Sjögren syndrome B are also present.are also present.


Diagnosis and Management of Diagnosis and Management of Sjögren SyndromeSjögren Syndrome

(pg. 100) (pg. 100) Diagnosis is made when two of three Diagnosis is made when two of three

components are present.components are present. XerostomiaXerostomia

• Measurement of salivary flow and biopsy can helpMeasurement of salivary flow and biopsy can help Keratoconjunctivitis siccaKeratoconjunctivitis sicca

• Confirmed with eye examinationConfirmed with eye examination Rheumatoid arthritisRheumatoid arthritis

For most patients, the course of the For most patients, the course of the disease is chronic and benign but these disease is chronic and benign but these patients are at risk for the development of patients are at risk for the development of other more serious diseases.other more serious diseases.


Diagnosis and Management of Diagnosis and Management of Sjögren Syndrome (cont.)Sjögren Syndrome (cont.)


Treatment of Sjögren SyndromeTreatment of Sjögren Syndrome

(pg. 100)(pg. 100) SymptomaticSymptomatic

Nonsteroidal antiinflammatory agents for Nonsteroidal antiinflammatory agents for arthritisarthritis• May need corticosteroids and immunosuppressive May need corticosteroids and immunosuppressive

drugs for severe casesdrugs for severe cases Saliva substitutes for xerostomiaSaliva substitutes for xerostomia

• Humidifier, sugarless gum, or lozengesHumidifier, sugarless gum, or lozenges• PilocarpinePilocarpine

Glasses and/or artificial tears to protect eyesGlasses and/or artificial tears to protect eyes


Systemic Lupus Erythematosus Systemic Lupus Erythematosus (SLE)(SLE)

(pgs. 100-102)(pgs. 100-102) An acute and chronic inflammatory An acute and chronic inflammatory

autoimmune diseaseautoimmune disease No known causeNo known cause

Affects women 8 times more frequently Affects women 8 times more frequently than men, predominantly during than men, predominantly during childbearing yearschildbearing years Three times more frequent in black women Three times more frequent in black women

than in white womenthan in white women


Systemic Lupus Erythematosus Systemic Lupus Erythematosus (SLE) (cont.)(SLE) (cont.)

A syndrome with a wide range of disease A syndrome with a wide range of disease activityactivity Usually chronic and progressiveUsually chronic and progressive

• Periods of remission and exacerbationPeriods of remission and exacerbation Autoantibodies to DNA are present in Autoantibodies to DNA are present in

serumserum May have a genetic componentMay have a genetic component


Clinical Features of Systemic Clinical Features of Systemic Lupus Erythematosus (SLE)Lupus Erythematosus (SLE)

(pgs. 101-102) (pgs. 101-102) Skin lesions occur in 85% of individualsSkin lesions occur in 85% of individuals

““Butterfly” rash on bridge of noseButterfly” rash on bridge of nose May be erythematous lesions on fingertipsMay be erythematous lesions on fingertips Arthritis and arthralgia are common.Arthritis and arthralgia are common.

Oral lesions accompany skin lesions in Oral lesions accompany skin lesions in about 25% of patients with discoid LE.about 25% of patients with discoid LE. Erythematous plaques or erosionsErythematous plaques or erosions

• May have white striae; resemble lichen planus but May have white striae; resemble lichen planus but are less symmetricare less symmetric


Clinical Features of Systemic Lupus Clinical Features of Systemic Lupus Erythematosus (SLE) (cont.)Erythematosus (SLE) (cont.)


Diagnosis of Systemic Lupus Diagnosis of Systemic Lupus Erythematosus (SLE)Erythematosus (SLE)

(pgs. 101-102)(pgs. 101-102) Usually based on multiorgan involvement Usually based on multiorgan involvement

and presence of antinuclear antibodies in and presence of antinuclear antibodies in serumserum Inflammatory infiltrate is around blood vessels Inflammatory infiltrate is around blood vessels

in connective tissue. in connective tissue.


Treatment and Prognosis of Systemic Treatment and Prognosis of Systemic Lupus Erythematosus (SLE)Lupus Erythematosus (SLE)

Aspirin and antiinflammatory drugs for mild Aspirin and antiinflammatory drugs for mild signs and symptomssigns and symptoms Hydroxychloroquine and corticosteroids along Hydroxychloroquine and corticosteroids along

with immunosuppressive agents may be used.with immunosuppressive agents may be used. The text recommends consultation with the The text recommends consultation with the

patient’s physician before initiating dental patient’s physician before initiating dental treatment.treatment.


Pemphigus VulgarisPemphigus Vulgaris

(pgs. 102-104)(pgs. 102-104) A severe, progressive autoimmune A severe, progressive autoimmune

disease affecting skin and mucous disease affecting skin and mucous membranesmembranes Characterized by intraepithelial blister Characterized by intraepithelial blister

formation resulting from acantholysis, a formation resulting from acantholysis, a breakdown of cellular adhesion between breakdown of cellular adhesion between epithelial cellsepithelial cells

Genetic and ethnic factors have been Genetic and ethnic factors have been reportedreported.. Often seen in Ashkenazic JewsOften seen in Ashkenazic Jews


Pemphigus Vulgaris (cont.)Pemphigus Vulgaris (cont.)

(pg. 103) (pg. 103) Oral lesionsOral lesions

The first signs of disease occur in the oral The first signs of disease occur in the oral cavity in more than 50% of cases.cavity in more than 50% of cases.• May be shallow ulcers, to fragile vesicles, to bullaeMay be shallow ulcers, to fragile vesicles, to bullae

Nikolsky signNikolsky sign• Rubbing with a finger can produce a bullae.Rubbing with a finger can produce a bullae.



(pgs. 103-104)(pgs. 103-104) Skin lesionsSkin lesions

Erythema, bullae, Erythema, bullae, erosions, ulcerserosions, ulcers

Microscopic Microscopic appearanceappearance Acantholytic cellsAcantholytic cells

• The loss of The loss of attachment attachment between epithelial between epithelial cells leads to cells cells leads to cells that appear that appear rounded.rounded.

Tzanck cellsTzanck cells


Diagnosis of Pemphigus VulgarisDiagnosis of Pemphigus Vulgaris

Made by biopsy and microscopic Made by biopsy and microscopic examinationexamination Direct immunofluorescenceDirect immunofluorescence

• Identifies autoantibodies present in tissueIdentifies autoantibodies present in tissue Indirect immunofluorecenceIndirect immunofluorecence

• The patient’s serum is used to detect circulating The patient’s serum is used to detect circulating autoantibodies.autoantibodies.


Treatment and Prognosis of Treatment and Prognosis of Pemphigus VulgarisPemphigus Vulgaris

High doses of corticosteroidsHigh doses of corticosteroids May include immunosuppressive drugsMay include immunosuppressive drugs Mortality rate of 8% to 10% in 5 years is Mortality rate of 8% to 10% in 5 years is

related to complications of corticosteroid related to complications of corticosteroid treatment.treatment.


Mucous Membrane Pemphigoid Mucous Membrane Pemphigoid (Cicatricial Pemphigoid) (BMMP)(Cicatricial Pemphigoid) (BMMP)

(pgs. 104-105) (pgs. 104-105) A chronic autoimmune diseaseA chronic autoimmune disease

Affects oral mucosa, conjunctiva, genital Affects oral mucosa, conjunctiva, genital mucosa, and skinmucosa, and skin

Not as severe as pemphigus vulgarisNot as severe as pemphigus vulgaris Gingival lesions have been called Gingival lesions have been called

desquamative gingivitis, but this may be seen desquamative gingivitis, but this may be seen with lichen planus and pemphigus as well.with lichen planus and pemphigus as well.

Will see positive Nikolsky signWill see positive Nikolsky sign


Mucous Membrane Pemphigoid Mucous Membrane Pemphigoid (Cicatricial Pemphigoid) (BMMP) (cont.)(Cicatricial Pemphigoid) (BMMP) (cont.)


Diagnosis of Mucous Membrane Diagnosis of Mucous Membrane PemphigoidPemphigoid

(pg. 104)(pg. 104) Made by biopsy and Made by biopsy and

histologic histologic examinationexamination No degeneration of No degeneration of

epithelium occurs epithelium occurs An inflammatory An inflammatory

infiltrate, usually with infiltrate, usually with predominant plasma predominant plasma cells and eosinophils, cells and eosinophils, is seen in connective is seen in connective tissue.tissue.


Treatment and Prognosis of Treatment and Prognosis of Mucous Membrane PemphigoidMucous Membrane Pemphigoid

A chronic disease with a benign courseA chronic disease with a benign course Topical corticosteroid for mild casesTopical corticosteroid for mild cases Systemic corticosteroids may be required for Systemic corticosteroids may be required for

more severe cases.more severe cases. Eye lesions can lead to eye damage.Eye lesions can lead to eye damage.


Bullous PemphigoidBullous Pemphigoid

(pg. 105)(pg. 105) Some investigators believe bullous and Some investigators believe bullous and

mucous membrane pemphigoid are mucous membrane pemphigoid are variants of a single disease, but 80% of variants of a single disease, but 80% of these patients are older than 60.these patients are older than 60. Oral lesions are less common than in cicatricial Oral lesions are less common than in cicatricial

pemphigoid.pemphigoid. TreatmentTreatment

Systemic corticosteroids and nonsteroidal Systemic corticosteroids and nonsteroidal antiinflammatory drugsantiinflammatory drugs


Behçet SyndromeBehçet Syndrome

(pg. 105)(pg. 105) A chronic, recurrent autoimmune diseaseA chronic, recurrent autoimmune disease

Primarily oral ulcers, genital ulcers, ocular Primarily oral ulcers, genital ulcers, ocular inflammationinflammation

No sex predilection; mean onset is 30 yearsNo sex predilection; mean onset is 30 years Autoantibodies to human mucosa may be Autoantibodies to human mucosa may be

found.found. Oral ulcers are similar in appearance to Oral ulcers are similar in appearance to

aphthous ulcers.aphthous ulcers.


Behçet Syndrome (cont.)Behçet Syndrome (cont.)


Diagnosis of Behçet SyndromeDiagnosis of Behçet Syndrome

Requires that two of three types of lesions Requires that two of three types of lesions (oral, genital, and ocular) be present.(oral, genital, and ocular) be present. A pustular lesion after needle puncture A pustular lesion after needle puncture

suggests Behçet syndrome.suggests Behçet syndrome.


Treatment and Prognosis of Treatment and Prognosis of Behçet SyndromeBehçet Syndrome

(pg. 105)(pg. 105) Systemic and topical corticosteroidsSystemic and topical corticosteroids

Chlorambucil is used for ocular lesions.Chlorambucil is used for ocular lesions.


Discussion Questions Discussion Questions

What is an autoimmune disorder?What is an autoimmune disorder? What is the difference between an antigen and What is the difference between an antigen and

an antibody?an antibody? What are the differences between active and What are the differences between active and

passive immunity?passive immunity? What oral diseases have an immunologic What oral diseases have an immunologic

pathogenesis?pathogenesis? What is an autoimmune disorder?What is an autoimmune disorder? What are the oral symptoms of Sjögren What are the oral symptoms of Sjögren

syndrome?syndrome? What are differences between pemphigus and What are differences between pemphigus and

pemphigoid? pemphigoid?

1 elsevier items and derived items © 2009 by saunders, an imprint of elsevier inc. chapter 3...

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