It includes autoimmune diseases affecting orofacial region n systemic autoimmune diseases showing oral manifestation.

1. AUTOIMMUNE
DISEASES OF ORAL
CAVITY
Guided By: Dr. Sandeep Sharma
(Oral Pathologist)
Presented By: Bijay Kumar Jaiswal
BDS, 6th Batch

2. Concept of
autoimmunity
 Autoimmunity is a condition in which structural or functional
damage is produced by the action of immunologically competent
cells or antibodies against the normal component of the body.
 Autoimmune diseases of oral cavity means autoimmune
disease or condition affecting orofacial region or showing
oral manifestation.

3. Role of a Dental Surgeon in
diagnosis of Autoimmune
Diseases.

4. 1.
2.
3.
4.
5.
6.
Endocrinal Autoimmune Diseases
Oral Autoimmune Diseases
Dermatological Autoimmune Diseases
Neural Autoimmune Diseases
Haematological Autoimmune Diseases
Multiorgan Involvement Autoimmune Diseases
A working classification by W.H.O
of autoimmune diseases based on
the organ involved is:

5. Autoimmune diseases affecting
orofacial region






Sjogrens syndrome
Benign lymphoepithelial lesion (mikulicz’s diseases)
Recurrent aphthous stomatitis
Oral lichen planus
Bechet disease
Psoriasis

6. Systemic autoimmune diseases
with oral manifestation






Pemphigus
Bullous phemigoid
Cicatrical pemphigoid
Systemic lupus erythematosus
Systemic sclerosis
(Scleroderma)
Idiopathic thrombocytic
purpura

8. Sjogren Syndrome (Sicca Syndrome,
Gougret-Syndrome)
Keratoconjunctivitis
Sicca
XerostomiaRheumatoid
Arthritis
Primary
Sjogren
Syndrome
Sjogren
Syndrom
e

9. Keratoconjunctivit
is sicca Secondary Sjogren
syndrome
SLE
Xerostomia Scleroderma
Polyarteritis
Nodosa

10. Etiopathogen
esis
Antigen Presenting Cells

12. What happens in Sjogren's
Syndrome then?




Infection of salivary and lacrimal gland
Damages the cell and exposes cell component like
DNA, RNA and histone
Picked by macrophage with T cell
Inappropriate activation of T cell and activate B cell

15. Clinical Features
Mainly due to loss of secretory acini as a result of immune
mediated damage.

16. Benign lymphoepithelial lesion
of the parotid gland causing

17. Histopathological Features


Intense Lymphocytic
infiltration but the
lobular architecture is
preserved.
Proliferation of Ductal
and myoepithelium to

18. Laborato
ry
findings
Polyclonal
hyperglobulinemia
Increased ESR
Tissues specific antibodies
– Antinuclear antibodies
(Anti­SSA and Anti­ SSB)

19. Radiographic findings
Cherry blossom or branchless fruit laden tree’ effect
radiographically.

20. Treatment and prognosis
Corticosteriod
Increases the
exocrine secretion
Pilocarpine
Ocular lubricants like
methylcellulose
(artificial tears)

21. Benign lymphoepithelial lesion
(Mikulicz Syndrome)



Chronic condition
Abnormal enlargement of salivary and lacrimal gland
Bilateral involvement
Etiolo
gy


Unknown
Suspected as autoimmune disorder
Symptoms are due to excessive
accumulation of lymphocyte into involve

22. Clinical
Features




Chronic dacryoadenitis
Recurring Fever (Differentiates
from Sjogren’s Syndrome)
Dry mouth, dry eyes
Lacrimal & Parotid gland
enlargement.

23. Histopathol
ogical
features



Lymphocytic
infiltration
Lobular
architecture is
destroyed
Epithelium shows
cellular
proliferation and
loss of polarity,
solid nest and

24. Differential Diagnosis





Metastatic carcinoma (epithelial island)
Malignant lymphoma (epimyoepithelial island not
present .Lymphoid element is atypical and infiltration of
interlobular septa by lymphoid tissue.)
Chronic sialadenitis
Papillary cystadenoma lymphomatosum
Uveoparotitis

25. Investigati
ons and
Treatment
Biopsy
USG
•
•
•
Artificial tears
(methylcellulose)
Artificial saliva
Follow soft and most diet
Symptomatic treatment

26. Recurrent Apthous
Stomatitis(Canker Sore)
Painful recurring solitary or multiple ulceration of oral mucosa






Precipitating factors:
Iron ,vitaminB12 or folic acid
deficiency
Trauma
Endocrine conditions
Psychic
Allergic
Systemic diseases

28. Classification


Recurrent Aphthous
minor- most common
Recurrent Aphthous
major- most severe
(Sutton diseases)

29. 

Recurrent
herpetiform
ulceration-consists
of clusters of ulcers
resembling herpetic
lesion
Recurrent ulcers
associated with
Behcets syndrome

30. 


Mucous membrane
exhibits fibro purulent
membrane covering
ulcerated area.
Intense inflammatory
infiltration in the
connective tissue beneath
the ulcer.
Epithelial proliferation is
present at the margin.
Histological
features

31. Differenti
al
Diagnosi
s
Herpetic stomatitis
Herpangina
Erythema multiforme
Erosive lichen planus
Pemphigus
Pemphigoid

32. Treatme
nt
Tetracycline
mouthwash(250mg per 5ml)
four times a day for 5­7days.
Steriod ointments
VitaminB12 and folic acid
supplement

33. Bechet's Syndrome
Autoimmune, multisystemic, chronic disorder, characterized by
oral and genital aphthous ulcers, arthritis, cutaneous lesion,
ocular, gastrointestinal and neurological manifestation.
Oral
ulcer
Genital
ulcer
Eye
inflammation
Bechet'
s
Syndro
me

34. Clinical
features





Age group 2nd -3rd decade
Site: Oral, genital, eyes and
skin.
Oral involvement is 25-75%
In oral involvement, soft
palate and oropharyngeal
areas are mostly involved.
Seen as an Apthous ulcer
with ragged border and
surrounded by larger zone
of diffuse erythema.

35. Histopathological
features



Similar to Apthous
ulcers.
Endothelial
proliferation is
seen in Bechet's
syndrome but not
in the recurrent
Apthous ulcer.
Fibrinoid necrosis
of vessel wall.

36. Laborato
ry
findings
Hypergammaglobulinemia
Elevated ESR
No diagnostic test is available
C­ reactive protein

37. Treatment
Symptomatic and
supportive measures
skin creams, oils
and oinments
Eye drops, mouth
rinse

38. Recurrent Aphthous
Stomatitis
Vs
Behcet’s Disease

39. 




Oral ulceration in recurrent aphthous stomatitis and in
Behcet diseases clinically indistinguishable.
In recurrent aphthous stomatitis, oral ulcer are only the
manifestation of diseases but in Behcet diseases at least
two of below manifestation is seen.
Recurrent oral ulcer
Recurrent genital ulcer
Ocular inflammation

40. Oral Lichen Planus(Lichen Ruber
Planus)




Most common mucocutaneous lesion.
Can affect either the skin or mucosa or both.
It can cause bilateral white striations ,papules, or plaque
on the buccal mucosa , tongue and gingiva.
Erythema, erosion may or may not be present.

41. ETIOPATHOG
ENESIS
•
•
•
•
Exact cause is unknown.
The lichen planus antigen is may
be self peptide.
Unmasking of antigen is
caused by drug, contact allergen
in dental restoration, mechanical
trauma , viral infection.
This antigen is associated with
keratinocyte.

42. 



CD8+ T cell recognize antigen and
binds with MHC-1 present on
keratinocyte.
CD8+ T cell activated ,releases
cytokines , trigger(start)
keratinocyte apoptosis.
More additional lymphocytes is
attracted towards developing lesion.
Due to which papule or
erythematous or ulcerated lesion is
formed.
ETIOPATHOG
ENESIS

43. Clinical features
•
•
•
•
Primarily effect the
middle aged adult,
more commonly
women.
Skin lesion of the
cutaneous form can
be described as 6Ps.
Pruritic, Polygonal,
Planar, Purple,
Papule and Plaque.
Its surface is
covered by
characteristic very
fine greasy white line
called Wickham’s
striae.

44. Oral
manifestation


1.
2.
3.
4.
5.
6.
Oral lesions are mainly
associated with the dermal
lichen planus but if only oral
lesion are present and none
elsewhere in the body, it is
called isolated lichen planus.
Lesion is mainly classified
into 6 types:
Reticular pattern
Plaque
Papular pattern
Bullous form
Erythematous form
Ulcerative form

45. Histopathological Features






Hyper: ortho- or
parakeratosis
Basal cell degeneration
Saw tooth rete ridges
Subepithelial band of
lymphocytic infiltrate
Civatte bodies may be
seen
Max-Joseph space

46. Differenti
al
diagnosis








Resemblance to reticular
lichen planus
Leukoplakia
Candidiasis
Lupus erythematosus
Resemblance to erosive
lichen planus
Pemphigus
Cicatricial pemphigoid
Erythema multiforme

47. TREATM
ENT
Corticosteroid.
Resolution of painful ,
oral mucosal lesion and
maintenance of good
oral hygiene.
If it is due to lichen
reaction , removal of
cause.

48. Psoriasi
s

Non contagious, chronic autoimmune disease .
Inflamed odematous skin lesion covered by silvery
white scales.
Etiology


Exact cause is unknown.
But may be genetic component, environmental trigger,
trauma or infection.

49. Pathogenesi
s
Then presented to Cytotoxic T cell and
get activated and release cytokines.
As dendritic cells are present on
spinosum layer, they engulf such
antigen.
Microbes are present on the skin surface.
Due to trauma or cut ,they enter
spinosum layer of epidermis

50. Pathogene
sis
Causes excessive inflammation and
leads to blood vessel of dermis to dilate
and more neutrophil to enter epidermis.
Recurite other immune cells like
neutrophils
Cytokine includes IL­12, IL­23,
Interferon­gamma, TNF, IL­17 (chronic
inflammation) and increase
keratinocyte proliferation.

51. Thins out stratum basale but thickens
corneum and spinosum layer.
This causes keratinocyte to proliferate
excessively and mature abnormally.
Neutrophil get collected in stratum
corneum
Pathogenesi
s

52. Scales picked off causes localized spot
of bleeding called AUSPITZ SIGN.
They retain nuclei (parakeratosis), do
not adhere properly (breaks in
epidermis and giving scaling
appearance)
This abnormal keratinocyte produce
more keratin
Pathogenesi
s

53. Clinical features




Age: 2nd -3rd decade
Characterized by occurrence
of small sharply delineated dry
papule and is covered by
delicate silvery scale
described as a thin layer of
mica.
Auspitz’s sign: If the deep
scale are removed one or
more bleeding points are seen.
Involvement of joints termed
as psoriatic arthritis.

54. Oral
manifestations





Oral psoriasis involved 4 type of
lesion:
Well defined yellowish white
lesion, round to oval in shape
White, lacy, circinate elevated
lesion on the mucosa and tongue.
Erythema and redness of entire
oral mucosa.
Geographic tongue.

55. Histological
Features
•
•
•
Uniform parakeratosis, absence of
stratum granulosum and elongation
and clubbing of rete pegs.
Intraepithelial Microabscess called
Munro Microabscess.
Tortuous dilated capillaries.

56. Treatment
Moisturizers and Emollients
to clear plaque and minimize
itchiness
Topical and systemic
immunosuppressive therapies
UV Phototherapy induces
DNA damage in
keratinocytes

57. Systemic
lupus
erythemat
osus




Systemic means affecting multiple
organs
Lupus means wolf (due to wolf bite
marks like rashes
Erythematosus means reddening
of skin
It is an autoimmune disease
characterized by autoantibodies,
immune- complex formation and
immune dysregulation resulting in
damage to essentially any organs

58. Etiopathogen
esis
Exact cause is unknown, but due to genetic
and environmental factor
Person having susceptible genes (genetic factor)
,smoking and under medication under UV rays
exposure(environmental factor)
Causes apoptosis of cell and part of nuclear
component gets exposed
Nuclear component like DNA, Histone and
other protein act as nuclear antigens as
recognized by body immune cells .
In person having susceptible genes, there is less
effective clearance of this nuclear antigen
Due to which nuclear antigen build up

59. Etiopathoge
nesis
B cell binds to these nuclear antigens and
produces antinuclear antibodies
Antinuclear antibodies(ANA) Binds to
nuclear antigen and form ANA antigen
complex and enters into blood stream
Through circulation antigen­antibody
complex deposited into different organ and
tissues like kidney, skin and joints
In localised inflammatory reaction,antigen­
antibody complex activate complement
systemEnzyme cascade and tissuedamage and cause
typeIII hypersensitivity

60. Clinical
Features

61. Oral
manifestati
ons

62. Histopathological
Features
•
•
•
•
•
Hyper: Ortho- or
parakeratosis
Keratotic plugging
and atrophy of rete
pegs
Liquefaction of
degeneration of
basal layer
Perivascular
infiltration of
lymphocyte
Follicular plugging

63. Laborator
y
Findings
•
•
•
ESR and CRP elevated.
Anti nuclear antibody (ANA)
C3 & C4 low in blood.

64. Treatmen
t



NSAIDs for Pain.
Steroid cream for rashes.
Antimalarial drugs for skin and joint
problems.

65. Pemphigus






Group of autoimmune blistering disease of skin and
mucous membrane characterized histologically by
intradermal blisters and immunologically by finding of
IgG antibody against the cell surface of keratinocyte
Subsets of Pemphigus
Pemphigus vulgaris
Pemphigus foliaceus
Paraneoplastic pemphigus
Familial benign pemphigus (Hailey-Hailey disease)

66. Normal Epithelial apparatus
Desmoglein1 and
desmoglien3

67. Etiopathogen
esis





In the genetically predisposed
people, the disease process is
triggered by herpes virus infection,
drugs like captopril, furosemide or
any other antibiotic.
Circulating IgG antibody is
produced against Dg1 and Dg3 (for
mucocutaneous lesion) and only
against Dg3 (for mucosal
pemphigus).
This causes apoptosis of the cells
and the release of protease
enzymes.
These enzymes break down the
Desmogellin protein.
This results in acantholysis, leading

68. Clinical
Features




Thin walled bullae of
varying diameter on a
normal skin or mucosa.
Nikolsky’s sign is positive.
Mean age 50-60 yrs.
Male : female = 1:1

69. Oral
Manifestations
•
•
•
Ill defined irregularly shaped
gingiva, buccal or palatal erosion
which are painful and slow to heal.
Erosion may spread to larynx
causing hoarseness of sound.
Uncommon variant, pemphigus
vegetans presents with
cerebriform tongue.

70. Histopathological
features



Intraepithelial split
or suprabasilar
split is seen.
Acantholysis is
seen forming the
characteristic cells
called Tzanck cells.
Tzanck smear
shows swelling of
nuclei and
hyperchromatic
staining.

71. Differenti
al
Diagnosi
s





Dermatitis herpetiform
Bullous pemphigoid
Erythema multiforme
Bullous lichen planus
Epidermolysis bullosa

72. Treatment



Corticosteroids- Systemic
prednisolone 1-2mg/kg/
day
Combination therapy of
high dose corticosteroid +
immunosuppressive drugs
such as cyclosporine,
cyclophosphamide
Others – Parenteral gold
therapy, Dapsone,
tetracycline

73. Pemphigus Foliaceus


It is also called Superficial pemphigus/ Brazilian
pemphigus/ Fogo selvagem.
Acantholysis is present forming superficial blister.
Pathogenesis


Abnormal IgG antibodies is produced against the Dg1
protein present in granular layer of epidermis.
This causes epithelial cell separation, acantholysis and a
suprabasilar bulla.

74. Clinical Features


Bullous lesion which
rapidly ruptures and
dries to leave a mass
of flakes forming
exfoliative dermatitis.
It is a mild endemic
form of pemphigus
found in tropical
region particularly in
Brazil.

75. Treatmen
t
•
•
•
•
•
•
•
Potent topical or intralesional
steroids
Oral prednisolone 20-40 mg/day
Azathioprine
Cyclophosphamide
Hydroxychloroquine 200md B.I.D
IV Ig
Dapsone 100mg/day

76. Pemphig
oid 2 types of Pemphigoid have been
reported:
Bullous
pemphigoid
Cicatricial
pemphigoid (Mucus
membrane
pemphigoid)
It is an autoimmune sub­epidermal
blistering disease.

77. 



Chronic, autoimmune, subepidermal, blistering skin
disease that involves the mucous membrane as well.
IgG antibody is produced against the hemidesmosomes
bullous pemphigoid antigens BP-230 (BP Ag1) and
BP-180 (BP Ag2).
Antigen antibody complex formation occurs activating
complement system.
More neutrophil and eosinophil accumulate releasing
protease enzymes causing breakdown of the
hemidesmosome protein leading to subepithelial blister
formation.
Bullous pemphigoid
(Parapemphigus)

78. Clinical Features
•
•
•
Age: Elderly above
60 yrs.
Site: Limbs and
Abdomen
predominantly
effected
Vesical and Bullae
doesn’t always
rupture, but when it
occurs, it leaves a
raw eroded area
which heals rapidly.

79. Oral
manifestations



Oral lesion occurs less in the
bullous pemphigoid than in the
cicatricial pemphigoid
Gingiva is the most common
effected site and is more painful.
Present as vesicle or bullae and
after rupture show painful erosion
and ulceration.

80. Histopatholo
gical
Features




Subepidermal and non-
specific vesical and
bullae.
Epithelial acantholysis is
absent.
Basement membrane
remains attached to
connective tissue.
Basement membrane
also shows thickening

81. Treatmen
t



Main aim of treatment is to decrease
blister formation and to promote
healing of blister and erosion.
Systemic Corticosteroids-
Prednisolone 0.75-1 mg/kg/day
Steroid-sparing agent- Azathioprine,
cyclophosphamide, Mycophenolate
mofetil

82. Cicatricial Pemphigoid



Also called benign mucous membrane pemphigoid or ocular
pemphigus.
Mainly effects mucous membrane including mouth, oropharynx
and conjunctiva.
IgG autoantibody is produced, mainly IgG4, against
hemidesmosomes BP-180 (BP Ag2) and epiligrin (laminin-5).

83. Clinical features
•
•
•
Site: Oral mucosa,
conjunctiva, genitalia,
nose and skin.
When there is a ocular
involvement, opacity of
cornea increases
leading to complete
blindness.
Peak age group: 40-50
yrs.

84. Oral
manifesta
tions




Gingiva is commonly
involved
Vesiculo-bullous in
nature and appears to be
thick walled
When ruptured, doesn’t
leave a raw eroded
bleeding surface
(differentiates from
bullous pemphigoid)
In the past it is

85. Differenti
al
diagnosi
s
Pemphigus vulgaris
Bullous pemphigoid
Ulcerative lichen planus
Bullous erythema multiforme

86. Scleroderma (Systemic
Sclerosis)




Generalized condition characterized by excessive collagen deposition
in skin and multiple internal organ, vascular alteration and
immunological abnormality.
Etiological Agent
Virus
Chemicals like silica
HLA-B8, DR5, DR52
Phenotypic changes in the cell

87. Pathogen
esis
1. Exact cause is unclear
2. Etiological agents when exposed to
genetically predisposed individuals,
leads to molecular and cellular
alteration.
3. Altered fibroblasts develop
excessive collagen in skin and other
organs.
4. Altered endothelial cell causes
obliteration of arteries and arterioles
5. Altered immune cell produces
dysregulated cytokines and auto
antibodies.

88. Pathogen
esis
5. When autoantibodies
combine with self antigen,
they form immune complex.
6. Immune complex along
with cytokines further
damage the blood vessels and
induce fibroblasts to produce
collagen.

89. Clinical
Features





Age: 30-50
Female predilection 3.6:1
Raynaud’s Phenomenon
when exposed to cold and
stress.
Skin is hard in texture and
can’t be wrinkled.
Claw like fingers.

90. Oro-facial
features





Face becomes expressionless,
mask like.
Elevated linear patches (Coup
de sabre) on face and trunk.
Microstomia (Purse string
appearance)
Tongue is stiff, hypomobile,
broad with difficulty in eating
and speaking.
Difficulty in swallowing.

91. Radiological
Features
•
•
Diffuse widening
of PDL space
Resorption of
posterior ramus,
coronoid process
and condyle in
10-20%.

92. Histopathol
ogical
Features

93. Treatme
nt
No satisfactory
treatment
Penicillamine to
reduce fibrosis
Corticosteroid Prognosis is poor

94. Idiopathic Thrombocytopenic
Purpura




Autoimmune disorder in which person becomes immune and
develops antibodies against his or her own platelets.
Two forms are seen:
Acute: Seen in children following a viral infection
Chronic: Seen in adults

95. Pathogen
esis
IgG antibody is produced against the
Gp2b3a present on the platelet surface.
Such antibody binding results formation
of antigen antibody complex.
This complex is engulfed by
macrophages of spleen leading to
destruction of platelets.
This causes low count of platelets in the
blood leading to thrombocytopenia.

96. Clinical Features



Purpuric or hemorrhagic lesion
is seen varying in size from tiny
red pin point petechiae to large
purplish ecchymosis.
Epistaxis, hematuria, Malena
may be present.
Bruising tendency is increased.

97. Oral Manifestations



Severe and profuse gingival
bleeding.
Petechial palate.
Ecchymoses.

98. Lab
Diagnosi
s



Platelet count is below 60,000/
cu.mm
Bleeding time is prolonged.
Coagulation time is normal.

99. Treatment