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AUTOIMMUNE DISEASES
OFTHE ORAL CAVITY
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Autoimmunity is the failure of an organism to recognize its own
constituent parts as self , which results in an immune response
against its own cells and tissues.
Greek word ‘auto’ meaning self.
Due to failure of an organism to recognize its own constituentparts as self leading to an immune response against its own cellsand tissues.
Body’s own immune system begins to attack normal tissues cellsand organs within the body.
AUTOIMMUNITY
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Autoimmune diseases are disorders in which the body'simmune system reacts against some of its own tissue and
produces antibodies to attack itself.
AUTO IMMUNE DISEASES
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Auto antibodies are the antibodies that attack its own cells,
tissues, and/or organs. This causes inflammation and damageand it leads to autoimmune disorders.
AUTO ANTIBODIES
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Distinguish self from non-self
Protect the body from foreign substances or pathogens
Hypersensitivity is an inappropriate, exaggeratedadaptive response that causes damage to the body
Reactions do not occur on the first contact
Gell and Coombs described four types
Functions of the Immune System
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Immediate hypersensitivity
IgE mediated
Target organs are mucosal surfaces of the GI tract,respiratory tract and conjunctiva
Allergic rhinitis, urticaria, atopic dermatitis, asthma, GIsensitivity.
Type I Hypersensitivity
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Antibody dependent cytotoxic hypersensitivity
IgG, IgM, NK cells and complement
Targets are circulating cells
Drug reactions, Goodpasture’s, pemphigus,myasthenia gravis, Lambert-Eaton, hemolytic diseaseof the newborn
Type II Hypersensitivity
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Immune complex –– mediated hypersensitivity
Antigen-antibody complexes (IgG)
Target organs include blood vessels of the skin, joints,kidneys, lungs.
Serum sickness, SLE, glomerulonephritis
Type III Hypersensitivity
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Delayed hypersensitivity
Cell-mediated (sensitized T lymphocytes)
Target organs include skin, lungs, CNS, thyroid
Three types:
Contact –– latex, nickel, poison ivy Tuberculin – 48 hours after PPD injection
Granulomatous leprosy, TB, sarcoidosis Crohn’s
Type IV Hypersensitivity
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Immune reaction against self-antigen
Range: single organ (cell) disorder to multisystem
Connective tissue or collagen vascular disease
Self-tolerance: no immune response to self
Clonal deletion: loss of T cell clones during maturation
Clonal anergy: inactivation induced by antigens
Peripheral suppression by T cells
Autoimmune Diseases
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Bypass of helper T-cell tolerance
Modification of molecule-drug complex
Costimulatory molecules (infection) Molecular mimicry
Microbes share epitopes with self-antigens
Streptococci and rheumatic heart disease
Polyclonal lymphocyte activation (Endotoxin, EBV)
Imbalance of suppressor/helper function
Emergence of sequestered antigens (e.g., eye, brain)
Mechanisms of AutoimmuneDisease (Loss of self-tolerance)
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AUTO-IMMUNE BULLOUS DISEASES OF THEORAL MUCOSA
EPIDERMOLYSIS BULLOSA LICHEN PLANUS
RECURRENT APTHOUS STOMATITIS
ERYTEMA MULTIFORME
SJOGREN’SSYNDROME
SCLERODERMA
WEGENER GRANULOMATOSIS
SYSTEMIC LUPUS ERYTHEMATOSUS
TYPES OF AUTOIMMUNE DISEASES
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Auto-immune Bullous Diseases of theOral Mucosa
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Bulla or Blister a (cavity) of the mucosa, containing serosity
or bloodi. situated inside of the epithelium : intra-epithelial bulla
ii. beneath the epithelium : sub-epithelial bulla
due to loss of cohesion and separation
i. between adjacent keratinocytes ( intra-epithelialbullae )ii. in the basement membrane zone ( sub-epithelial bullae )
Bullae are different from
I. vesicles : smaller, due to necrosis or collected oedema
II. pustules : variable size and structure, and contain pus
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intraepithelial bulla subepithelial bulla
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Autoimmune disease.
Common in Ashkenazi and Mediterranean jews .
Middle aged females.
Other variants are:
Pemphius VegitansPemphigus Foliaceus & Erthematosus
Paraneoplastic pemphigus.
PEMPHIGUS VULGARIS
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CLINICAL FEATURES:
Painful ulcers or bulla are formed which are fluidfilled.
They can be formed any where in the oral cavity .
The bulla is rapidly ruptured leaving a collapsed roof
of grayish membrane with a red ulcerated base.Theulcer may look like an apthous ulcer or may be largemap shaped.
Nikolsky sign is positive.
PEMPHIGUS VULGARIS
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Some time the ulcers are joined together to make
a confluence this condition is very painful. It has a variable course might involve skin,
oesophagus, cervix.
Protein/fluid,electrolyte and weight loss/secondary infections.
Fatal if untreated.
PEMPHIGUS VULGARIS
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PEMPHIGUS VULGARIS
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Pemphigus vulgaris
Severe, potentially fatal Jewish and Italians
Intraepithelial bullae and acantholysis
Nikolsky’s sign
Loss of intracellular bridges Autoimmune response to desmoglein 3
Intraepithelial clefting
Pemphigus vulgaris
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Pemphigus vulgaris
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PATHOGENESIS:
It is an autoimmune disease There are circulating antibodies of type IgG. These antibodies are reactive against the
desmosomes or the tonofilament complex.
There destruction or disruption of thesetonofilament complex ,resulting in the loss ofattachment from cell to cell
PEMPHIGUS VULGARIS
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The epithelial damage is directly proportion to
the number of the circulating antibobies. The tonofilament or desmosomes are
disrupted by a proteolytic enzyme which isreleased by these antibodies .
The cell to cell break down also takes placethrough a complement system but thisprocess is not clearly understood .
PEMPHIGUS VULGARIS
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PEMPHIGUS VULGARIS
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HISTOPATHOLOGY: Intra epithelial vesicles or bulla and cleft like spaces are produced
by acantolysis These changes are in the stratum spinosum or the prickle cell
layer The basal cell remain attach to the lamina propria and project
into the bulla like tombstones. Inflammatory cells are very scanty however eosinophils may be
seen. Acantholytic statum spinosum cells occur singly or are in the
forms of clumps lying freely within the blister fluid. These cellloose there polyhedral morphology rather they are smallrounded and contain hyper chromatic nuclei called the TAZANKCELLS.
PEMPHIGUS VULGARIS
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PEMPHIGUS VULGARIShistology
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PEMPHIGUS VULGARIShistology
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PEMPHIGUS VULGARIStazank cells
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PEMPHIGUS VULGARISimmunoflorecence
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DIFFRENTIAL DIAGNOSIS:
Pempegiod
Erthema multiforme
Bullous lichen plannus
PEMPHIGUS VULGARIS
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TREATMENT:
High mortality rates previously Introduction of systemic corticosteroids like
prednisolone in stable cases.
Prednisolone plus azathioprine methotrexate and
cyclophospamide in progressed or advance cases.
PEMPHIGUS VULGARIS
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PEMPHGOID
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PATHOLOGY Autoimmune disease Not life threatening
Elderly females above 60 yrs of age Loss of attachment and separation of full thickness
epithelium from the lamina propria. Alteration of rete pegs Epithelium forms the roof of the blisters Auto antibodies are formed against the
hemidesmosomes (BPAG-1,230kd;BPAG-2; 180kd. Inflammatory
cells(lymphocytes,neutrophils,eosinophils)are seen inthe later stages
PEMPHGOID
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Mucous membrane pemphigoid (cicatricial) CIKA-
TRI-CIAL Bullous pemphigoid
PEMPHGOID
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Bullous pemphigoid (BP) is a rare autoimmune subepidermal bullous diseaseprimarily affecting the elderly population after 60 years of age. Males are
equally as affected as females. In many cases, the cause of BP is suspectedto be medications. BP is mediated by the formation of autoantibodies binding to bullous
pemphigoid antigens 230 and 180, cytoplasmic and transmembrane portionshemidesmosomes of basal cells in the epidermis.
IgG autoantibodies are found in circulation and bound to the lamina lucidalayer of the basement membrane. These antigen-antibody complexes
trigger the release and activation of complement with leukocyte chemotaxisand subsequent degranulation. The release of proteolytic enzymes resultsin the degradation of the BMZ with separation of the epidermis from thedermis.
Bullous Pemphigoid
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The presentation of BP is commonly oral blisters (24%), and is usuallytransient.
Initially there may be a localized erythematous plaque which may bepruritic, and subsequently enlarges with edema to become tense bullae. These lesions are usually generalized an most commonly affecting the lower
abdomen, groin, and flexor surfaces. There is a negative Nikolsky sign. These bullae usually rupture in a week, which leaves a localized are of
erosion which heals quickly. There are multiple variants of BP with vesicular, vegetating, hyperkaratotic,
and erythrodermic appearances. However, they all share the samehistologic and immunologic characteristics of BP.
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I. The diagnosis depends on skin biopsy.
II. A subepidermal cleft with the present of eosinophils in the dermis and
bullous regions are common histologic findings.III. Direct immunofluorescence indicates the deposition of IgG, and/or C3,
and variably IgA, IgM, and fibrin in a linear fashion at the BMZ.
IV. Indirect immunofluorescence is needed to differentiate BP from otherbullous diseases.
V. Circulation IgG antibodies targeting the BP230 and BP180 antigensfound in BP.
VI. Mortality at 1 year is near 19% with treatment.
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Treatment of BP is dependent on the extent and severityof disease.
Tetracycline, minocycline, or erythromycin with orwithout niacinamide has indicated excellent clinicalresponse for localized and generalized disease.
Topical steroid therapy has been to be effective for all
forms of BP and is superior to oral corticosteroids. Clobetasol proprionate cream (0.05%) has been
effectively used as a topical agent in treating BP.
Prednisone (0.5-1 mg/mg/daily) may be used ingeneralized BP
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Mucous membrane pemphigoid (MMP), or cicatricialpemphigoid, is a rare chronic immune-mediated diseasecharacterized by blistering, ulcers, and scarring.
This disease usually affects adults from the age of 40 to 60and there is found in twice as often in woman than men.
It results from the production of autoantibodies againstantigens within the basement membrane zone of the laminalucida.
Mucous Membrane Pemphigoid(Cicatricial Pemphigoid)
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CLINICAL FEATURES(MMP)
Oral mucosa is the first site- lesions are rarelywide spread Subepithelial bullae, ruptured in the later stages. Bleeding in the bullae – bleeding blisters
Slow progress, skin involvement absent or rare Involvement of eyes, nose larynx, pharynx and
osephaghus Nikolsky sign is positive
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Diagnosis of MMP is established on biopsy taken from the lesionedge that includes ulcerated and nonulcerated portions.
Hematoxylin and eosin staining typically indicates separation ofthe mucosal epithelium from the underlying tissue at the level of
the lamina lucida between the basal cell layer and the laminadensa.
Definitive diagnosis requires clinical correlation with directimmunofluorescence findings.
There are linear deposits of one or a combination of IgG, IgA, andC3 at the basement membrane zone in a continuous andhomogeneous pattern.
MMP may be distinguished histologically from pemphigus by the
location of blisters.
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Treatment for MMP may be treated topically by debridement ofnecrotic tissue and oral rinses of hydrogen peroxide,
Elixir of dexamethasone, and elixir of diphenhydramine (diluted1:4 or 1:6 with water.
Before meals, hydrogen peroxide rinse with diphenhydraminemay be used for cleaning and reducing pain.
Fluocinonide gel is an alternative and is adherent to ulcers withbetter patient compliance than triamcinolone in Orabase.
A soft acrylic appliance may be used when there is gingivalinvolvement.
Intralesional steroids may be injected into oral cavity lesions
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CASCADEOF EVENTSAntibody antigen complex
Complement activation
Neutrophils & Eosinophils recruited
Release of proteases by the recruited cells
Sub epithelial blister formation
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MANAGEMENT
Confirm diagnosis Topical corticosteroids
Ocular involvement –systemic steroids.
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EPIDERMOLYSIS BULLOSA
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Definition:
A large group of clinically similar desquamatingdisease processes of the skin and mucosa that have incommon the separation of the epithelium from theunderlying connective tissue and the formation oflarge blisters that frequently result in extensive and
often immobilizing scar formation.
EPIDERMOLYSIS BULLOSA
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MAJOR CATEGORIES OF EPIDERMOLYSIS BULLOSA
Type Genetic Pattern Separation Level Defec. Structure
Hereditary
Simplex Autosomal dominant Intraepithelial linking proteins
Junctional autosomal recessive lamina lucida anchoring filaments
Dystrophic autosomal dominant sublamina densa type VII collagen
Acquired
Acquisita None/autoimmune sublamina densa type VII collagen
EPIDERMOLYSIS BULLOSA
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HEREDITARY TYPES:
Congenital absence of components
ACQUIRED TYPES:
Autoantibodies (IgG; sometimes IgA) to type VIIcollagen.
EPIDERMOLYSIS BULLOSA
EPIDERMOLYSIS BULLOSA
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EPIDERMOLYSIS BULLOSA
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CLINICAL FEATURES
1. Epidermolysis Bullosa Simplex Mild form; autosomal dominant
Sites of trauma/friction
Involve hands, feet and neck; occ. knees and elbows
Teeth not affected; intraoral blisters seen Appears during infancy
EPIDERMOLYSIS BULLOSA
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EPIDERMOLYSIS BULLOSA
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2. Junctional Epidermolysis Bullosa
Severe form; autosomal recessive Haemorrhagic blisters; loss of nails, large blistersof face, trunk and extremities
Generalized scarring and atrophy Intraorally-haemorrhagic blisters of palate,
perioral and perinasal areas Erupted teeth exhibit hypoplastic and severelypitted enamel prone to caries
EPIDERMOLYSIS BULLOSA
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3. Dystrophic Epidermolysis Bullosa Both autosomal dominant and recessive; recessive is
severe Lesions are birth; arise at pressure sites Blisters rupture leaving painful ulcers which heal with large
scars that undergo contractures, leading to loss of motilityand claw-like hands (Mitten Deformity)
Teeth exhibit delayed eruption and enamel hypoplasia withrapid caries development
Scarring around mouth leads to diminished opening,ankyloglossia
EPIDERMOLYSIS BULLOSA
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Epidermolysis Bullosa Acquisita
Non-hereditary form; appears in adulthood Clinically resembles autosomal dominant type of JEB-
type VII collagen
Trauma/friction induced blisters of knees, elbows,
hands and feet- heal with scars Intraoral blisters rare- when present same picture
same picture as JEB
EPIDERMOLYSIS BULLOSA
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HISTOPATHOLOGY
Simplex type exhibits zone of cleavage (intra-epithelial) above basal cell layer.
Remaining types have sub-epithelial separation
EPIDERMOLYSIS BULLOSA
EPIDERMOLYSIS BULLOSA
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EPIDERMOLYSIS BULLOSA
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MANAGEMENT No specific treatment available for hereditary
types Acquired form maybe treated with
corticosteroids and immuno-suppressants Maintenance of pt’s nutritional and oral hygiene
status
Wound healing techniques Prevention of infections Systemic use of Phenytoin (also acts as a
collagenase inhibitor)
EPIDERMOLYSIS BULLOSA
Li IgA Di
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Linear IgA is an acquired blistering disorder without a
definitive cause. There are two clinical types:
I. chronic dermatosis of childhood occurs in the firstten years,
II. adult linear IgA disease occurring later with peak
between 60 to 65 years.
(Note: These forms share the same histologic and immunologic findings,and may share the same target antigen.)
Linear IgA Disease
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There are twice as many females affected by this disease than males,and may affect any skin site.
The lesions may be painful and pruritic.
Elevated erythrocyte sedimentation rate and circulating IgA may bepresent.
This blistering disorder has a tendency to occur in the trunk, limbs, face,perioral region, and hands.
This may clinically mimic dermatitis herpetiformis (also has IgA deposits),bullous pemphigoid, or other bullous diseases.
The oral mucosa is also affected and results in scarring.
This may resemble the presentation of desquamative gingivitis. Vesicularlesions may become confluent and form large bullae, and finally burst
leaving ulcerated areas.
Clinical Features
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There are multiple antigens involved in linear IgA disease,
and the antibodies bind to multiple sites on a single antigen. The bullous pemphigoid antigen BP180 and its extracellular
domain LAD1 are most often implicated in the diseaseprocess.
Gluten sensitivity has been found in one quarter to one thirdof affected patients.
Other associated diseases include rheumatoid arthritis,ulcerative colitis, immune glomerulonephritis, andmalignancy including lymphoma.
Findings
Linear IgA Disease
Linear IgA Disease
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Diagnosis of linear IgA disease depends upon biopsy ofperilesional areas with immunofluorescence.
Histologic appearance may indicate microabscesses andinfiltration of eosinophils in the superficial corium.
There may also be few lymphocytes present surroundingsmall vessels.
A homogeneous deposition of IgA, and possibly C3, is presentalong the basement membrane zone detected using direct
immunofluorescence in skin biopsy. This is in contrast to dermatitis herpetiformis, where IgA
deposits are present usually at the tips of connective tissuepapillae.
Diagnosis
Linear IgA Disease
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Treatment of linear IgA disease may require combinationtherapy.
Topical and systemic steroids often do not effectively controlthis disease alone.
Dapsone, adult dosing 25-150 mg daily, is effective in most casesfor controlling symptoms of burning and itching,
TreatmentLinear IgA Disease
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Lichen Planus
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Lichen Planus
Lichen planus is an idiopathic inflammatory disorder involving
the skin and mucous membranes. The age of onset is about 40 years in men, and 46 years in
women. It is rarely found under the age of 5 years.
There is positive family history in 10% of patients, and an
increased frequency of HLA-B7 has been associated. There may be an association with hepatitis C virus.
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variable and present as white striations (Wickhamstriae), white papules, white plaques, erythema(mucosal atrophy), erosions (shallow ulcers), orblisters.
The lesions predominantly affect the buccalmucosa, tongue, and gingivae, although otheroral sites are occasionally involved.
a T-cell–mediated autoimmune disease in whichautocytotoxic CD8 + T cells trigger the apoptosisof oral epithelial cells
Slightly increased risk of oral SCCa
Lichen Planus
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Chronic disease of skin and mucous membranes
Destruction of basal cell layer by activatedlymphocytes
Reticular: fine, lacy appearance on buccal mucosa(Wickman’s striae)
Hypertrophic: resembles leukoplakia
Atrophic or erosive: painful
Lichen Planus
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Lichen Planus
1. Spider web.
2. The buccal mucosa involved most often
3. reticular form most common
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Reticular Oral Lichen Planus
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Lichen planus
Lichen Planus
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Lichen Planus
A very high power viewof the dermoepidermaljunction
Civatte bodies (arrows),
keratinocyteenlargement, andcoarse collagen bundles
are illustrated.
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Lichen planus (LP) is a self-limited disease that usuallyresolves within 8-12 months.
Mild cases can be treated with fluorinated topical steroids.
More severe cases, especially those with scalp, nail, andmucous membrane involvement, may need more intensivetherapy.
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ERYTEMA MULTIFORME
ERYTEMA MULTIFORME
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Mucocutaneous disease
Males adolosents , young adults are affected more
ERYTEMA MULTIFORME
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ERYTEMA MULTIFORME
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AETIOLOGY /PATHOLOGY
Unclear aetiology and pathogenesis Infections like HSV can trigger this disease
Drugs like Sulphonamides ,barbiturates
Suggested cause is also given as to a type III
hypersensitivity reaction
ERYTEMA MULTIFORME
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CLINICAL FEATURES
Prodomal signs: Upper respiratory infection
Headache and malaise
Nausea and arthralgia
ERYTEMA MULTIFORME
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Signs during the disease: Red macules – 1cm or more in diameter with
cyanotic center Lips grossly swollen ,split crusted bleeding Widespread fibrin covered erosions and erythema
in the mouth.
Mild fever Conjunctivitis may be associated Attacks recur at the intervals of several months Usually self limiting.
ERYTEMA MULTIFORME
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ERYTEMA MULTIFORME
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HISTOPATHOLOGY
Necrosis of the kertinocytes Inter & intra cellular odema.
Subepithelial blisters are common
Infiltration of inflammatory cells.
ERYTEMA MULTIFORME
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ERYTEMA MULTIFORME
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MANAGEMENT
No specific treatment required , if HSV inf.. acycovir
Systemic steroids may give relief to the fever.
In severe cases antibiotics are used to prevent ant secondaryinfections.
Symptomatic –analgesics, antipyretics, antihistamines.
ERYTEMA MULTIFORME
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RECURRENT APTHOUSSTOMATITIS
( )
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Most common ulcerative lesion of oral cavity
Recurrent, painful ulcers Confined to soft mucosa
Subdivided into three types: Minor aphthae
Major aphthae
Herpetiform aphthae
Recurrent Aphthous Stomatitis(RAS)
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ETIOLOGYThe etiology is not basically understood but increasing
evidence of damaging immune response is being given.How ever some of the factors are considered to be thecause.1.Immunological factors2.hereditary factors3.Microbiological factors4.Emotional stress5.Nutritional deficiencies6.Allergic disorders.7.Hematalogical factors.8.Gastrointestinal factors
Recurrent apthous stomatitis
h h i i ( )
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CLINICAL FEATURES Minor aphthae:
Heal completely in 7-10 days without scarring
Painful
Prodromal stage
Gray to yellow membrane
Clusters of up to 5 ulcers
Steroids
May be shallow and round effecting the non-keratinized part of the oral epithelium
Diameter of the ulcers is less than 10mm with red margins.
Site is usually the tongue, buccal mucosa soft palate floor of the tongue
Recurrent Aphthous Stomatitis(RAS)
h h i i ( )
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Minor apthae
Recurrent Aphthous Stomatitis (RAS)
Recurrent apthous stomatitis
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Recurrent apthous stomatitisminor apthous ulcers
R h i i
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MAJOR APTHOUS ULCERS
Larger than the minor apthous ulcer diameter more than 10mm.
Site similar to that of the minor apthous ulcers.
Also involve the keratinzed part of the epithelium.
They vary in number from 1-10.
Take 4-6 weeks to heal. Heal with scarring.
Recurs in less than a months time.
Recurrent apthous stomatitis
R A h h S i i (RAS)
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Major apthae
Recurrent Aphthous Stomatitis (RAS)
Recurrent apthous stomatitis
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pmajor apthous ulcers
R t th t titi
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HERPETIFORM ULCERATION
Multiple small pinhead size .Each ulcer1-2 mm in size.
Can occur at any part of the oral cavity and as many ashundreds of small ulcers may be present.
The ulcers are present in the form of clusters or corps andsometime these are joined together to form a very largeulcer.
They also heal with scarring.
Recur in less than a month time.
Associated with extreme pain and discomfort.
Recurrent apthous stomatitis
Recurrent apthous stomatitis
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Recurrent apthous stomatitisherptiform ulcer
R t th t titi
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HISTOPATHOLOGY(minor ,major, herptiform)
Mononuclear cells are found in the submucously in the preulcerative stage
These mononuclear cells are the T-4 lymphocytes and are soon
out numbered to T-8 lymphocytes as the ulcerative stagedevelops.
Macrophages and the mast cells are also present at the baseof the ulcer.
Recurrent apthous stomatitis
R t th t titi
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TREATMENT(Minor,Major,herptiform) Minor apthous ulcers require no treatment only topical
gels are used to minimize the pain ,as the ulcer is selflimiting and heals with in7-10 days Anti inflammatory gels and mouth washes are also used to
prevent any further infection and to control theinflammation caused by the ulcer
For major apthous ulcer topical corticosteriods may be
used In extreme severe cases systemic steroids such as
prednisilone in doses of 20-40mg daily have shownpromise
Recurrent apthous stomatitis
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Sjogren’s Syndrome
Sjogren’s Syndrome
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Systematic, autoimmune disorder
Occurs in association with disorders such as
Rheumatoid arthritis Systemic lupus erythematosus: is a systemic autoimmune disease that can
affect any part of the body
Scleroderma:is a chronic systemic autoimmune disease characterized byfibrosis (or hardening), vascular alterations, and autoantibodies.
Polymyositis: many muscle inflammation.
Polyarteritis: a serious blood vessel disease in which small andmedium-sized arteries become swollen and damaged
Sjogren’s Syndrome
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Keratoconjunctivitis sicca (dry eyes)
Xerostomia (dry mouth)
Immune-mediated destruction of lacrimal and salivary glands
Primary: “sicca syndrome” HLA-DR3
Secondary: with RA, SLE, etc. HLA-DR4
Women >40 yo
B cell lymphoma in 1%
Pseudolymphoma in 10%
Sjogren s Syndrome
Sjogren’s Syndrome
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j g y
xerostomia and xerophthalmia
Sjögren’s Syndrome
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Chronic autoimmune disorder
Major clinical manifestations resulting from changes inexocrine glands
Forms of Sjögren’s Syndrome
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Primary Sjögren’s is characterized by inflammatory cellinvolvement of both the salivary and lacrimal glands
Secondary Sjögren’s includes other defined connectivetissue disease
Causes are unknown
Features of Sjögren’s Syndrome
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Glandular epithelial cells participate in the autoimmune
disease process
Epithelial cells produce a number of immunologically
active mediators
May serve as antigen-presenting cells
Epithelial cell responses modulate mechanisms occurring in
the salivary glands
Is Sjögren’s Syndrome an Autoimmune
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Described as an autoimmune exocrinopathy (Strandand Talal, 1980)
Grouped with other connective tissue diseases
Rheumatoid arthritis
Systemic lupus erythematosis (SLE)
What is the evidence that it is an autoimmunedisease?
gDisorder?
Evidence that Sjögren’s Syndrome is an
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A specific auto-immunogen and pathogenic
antibodies have not been identified Autoantibodies that have been found have
not been shown to have any directpathogenic effects on exocrine tissues
There is substantial circumstantial evidencethat tissue damage is the result ofautoimmunity
Autoimmune Disease
Polyclonal Hypergammaglobulinemia
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B-cell hyper-responsiveness
Marked elevations of IgG Production of rheumatoidfactors
Presence of anti-nuclear antibodies Extractable nuclear antigens Anti-SS-A (Ro) and anti-SS-B (La)
Antibodies are found directed against salivary duct
cells (90% of patients) Primarily against extractable nuclear antigens
Concentration does not correlate with gland destruction
Polyclonal Hypergammaglobulinemia
Other Characteristics
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Elevated sedimentation rates and decreased WBCcounts, as seen in other autoimmune connectivetissue diseases
Specific extended MHC haplotype at a higherfrequency than controls
MHC-encoded proteins
Induction of tolerance to self proteins Selection of the T-cell repertoire Binding and presentation of antigen to T-cells
Other Characteristics
Histopathology
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Mononuclear infiltrate consisting primarily of
T-cells (primarily CD4+
) Host of mediators
Altered cell adhesion molecules expression
Increased HLA class II antigens expression Immunosuppressive therapy often effective
Histopathology
Classical Histopathological Lesion
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Lympho-epithelial lesion affecting the parotidgland
Progressive replacement of the salivary tissue bydense lymphoid infiltrates
Formation of proliferating islands of ductalepithelial cells
Creates well-formed lymphoid follicles typical ofMALT and may give rise to lymphomas of theMALT type as an expansion of monoclonal B-cells
Classical Histopathological Lesion
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SalivaryGland
Structure
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Conclusion
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Numerous changes in immune factors in
Sjögren’s Syndrome Salivary glands appears as a highly active,
immune-mediated inflammatory sites
Salivary epithelial cells are immunologically-active participants in the disease process
Conclusion
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WEGENER
GRANULOMATOSIS
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Definition and etiology
Wegener granulomatosis is a rare chronic granulomatousdisease with a probably immunological pathogenesis
Wegener granulomatosis: large ulcer surrounded by an
erythematous zone on the tongue.
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Clinical features
• The disease is characterized by necrotizinggranulomatous lesions of the respiratory tract,generalized focal necrotizing vasculitis, and necrotizing
glomerulitis.• The oral lesions are fairly common, and present clinically
as solitary or multiple irregular ulcers, surrounded by aninflammatory zone
• The tongue, palate, buccal mucosa, and gingiva are themost commonly affected areas.
• characteristic hyperplastic gingivitis (strawberrygingivitis).
• heavily inflamed granular exophytic lesions• deep necrotic oral ulceration.
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Laboratory tests
Histopathological examination, detection of antineutrophilcytoplasmantibod ies (ANCA) in the serum.
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Differential diagnosis
Malignant granuloma,
tuberculosis,
non-Hodgkin lymphoma,
leukemia,
systemic mycoses,
squamous-cell carcinoma.
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Treatment
A combined regimen with steroids, azathioprine, andcyclophosphamide.
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Scleroderma
Scleroderma
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Scleroderma
Definition
Scleroderma is a progressive disease that affects theskin and connective tissue.
There are two major forms of the disorder.i. The type known as localized scleroderma mainly
affects the skin.
ii. Systemic scleroderma, which is also called systemicsclerosis, affects the smaller blood vessels andinternal organs of the body.
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Description
Scleroderma is an autoimmune disorder, which meansthat the body's immune system turns against itself.
In scleroderma, there is an overproduction ofabnormal collagen (a type of protein fiber present inconnective tissue).
This collagen accumulates throughout the body,causing hardening (sclerosis), scarring (fibrosis), andother damage.
Scleroderma
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Scleroderma
Raynauds phenomenon is seen as the first symptom.
Chronic multi-system disease characterized by diffuse fibrosis of skin and internalorgans.
Facial involvement results in restricted mouth opening & expressionless (mask-like)face.
Females aged 20-50 years.
Generalized widening of PDL space on oral radiographs.
Association with other autoimmune diseases : LE, RA, Sjögren’s syndrome suggestsautoimmune etiology.
Resricted Mouth opening is another major problem.
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Treatment A drug called D-penicillamine has been used to interfere with the
abnormal collagen.
Taking vitamin D and using ultraviolet light may be helpful for localizedscleroderma.
Corticosteroids have been used to treat joint pain, muscle cramps, andother symptoms of inflammation.
The various complications of scleroderma are treated individually.
Raynaud's phenomenon requires that people try to keep their handsand feet warm constantly. Nifedipine is a medication that is sometimesgiven to help control Raynaud's.
Thick ointments and creams are used to treat dry skin. Exercise and massage may help joint involvement; they may also help
people retain more movement despite skin tightening. An exerciseregimen for stretching the mouth opening has been reported to be ahelpful alternative to surgery in managing this condition.
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Diagnosis
Diagnosis of scleroderma is complicated by the fact that
some of its symptoms can accompany other connective-tissue diseases. The most important symptom is thickened or hardened
skin on the fingers, hands, forearms, or face. The person's medical history may also contain important
clues, such as exposure to toxic substances on the job. There are a number of nonspecific laboratory tests onblood samples that may indicate the presence of aninflammatory disorder.
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Systemic Lupus Erythematosus
Systemic Lupus Erythematosus
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y p y
Definition
Lupus erythematosus is a chronic immunologically mediateddisease.
Systemic Lupus Erythematosus Criteria (4or more)
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or more)
1. Butterfly rash
2. Discoid lupus
3. Photosensitivity4. Oral ulcers
5. Arthritis
6. Serositis
7. Neurologic dis
8. Hematologic dis
9. Renal disorder10. Immunologic dis: LE cell,
anti-DNA, anti-Sm, falsepos STS
11. Anti-nuclear antibody
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Incidence 1:2500
Female: male 10:1
2nd/3rd decade of life
More common and severe in Blacks Skin, kidney, serosal membranes, joints, heart
Many autoantibodies
Failure to maintain self-tolerance
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Etiology Autoimmune.
Clinical features
•Two main forms of the disease are recognized: discoid (DLE)and systemic (SLE).
•Oral lesions develop in 15–25% of cases in DLE and in 30–45% of cases inSLE, usually in association with skin lesions.
•The oral lesions are characterized by a well-defined central atrophic
red area surrounded by a sharp elevated border of irradiating whitishstriae (Fig. ).•Telangiectasia, petechiae, edema, erosions, ulcerations, and white
hyperkeratotic plaques may be seen.
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Laboratory tests
1. Histopathological examination2. Direct immunofluorescence.
Differential diagnosis
I. Lichen planus,
II. geographic glossitis,
III. Speckled
IV. leukoplakia,
V. erythroplakia,
VI. cicatricial pemphigoid,
VII. syphilis.
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Treatment
1. Steroids,
2. antimalarials.