2. MUCOCUTANEOUS
DISORDERS
OF
ORAL CAVITY
GENODERMATOSES INFECTIVE
NON-
INFECTIVE

3. GENODERMATOSES
• White sponge nevus
• Darrier’s disease
• Peutz-Jeghers syndrome
• Dyskeratosis congenita
• Hereditary benign interepithelial
dyskeratosis
• Panchyonychia congenita
• Hyalinosis cutis et mucosa oris
• Pseudoxanthoma elasticum

4. INFECTIVE
• Primary herpetic ginigivostomatitis
• Secondary herpetic ginigivostomatitis
• Varicella ( chickenpox)
• Shingles ( Herpes zoster)
• Measles
• Hand- foot-mouth disease
• Small pox and cat scratch disease
• Infectious mononucleosis
• Herpangia
• Acute lymphonodular phayngitis
• AIDS

5. NON-INFECTIVE
A.VESICULAR
B.NON-VESICULAR
C.COLLAGEN DISORDER
D.DEGENERATIVE AND
RELATIVE DISORDER
E.PIGMENTATION

6. A. VESICULAR
• Erythema multiforme
• Pemphigus
• Benign mucous membrane
pemphigoid
• Bullous pemphigoid
• Epidermolysis bullosa
• Bullous lichen planus

7. B. NON VESICULAR
•Lichen planus
•Benign migratory
glossitis

8. C. COLLAGEN DISORDER
• Lupus erythematous
• Scleroderma
• Polyarteritis nodosa
• Vasculitis
• Ischemic lingual necrosis
• Wegner granulomatosis
• Midline lethal granuloma

9. D.DEGENERATIVE AND RELATIVE
DISORDER
•Amyloidosis
•Oral submucous fibrosis
•Senile solar elastosis

10. E . PIGMENTATION
•Racial pigmentation
•Endocrinopathy
•Addison disease
•Albert syndrome
•Bronze diabetes
•Anemia

12. ERYTHEMA MULTIFORME(EM)
• Erythema multiforme is an acute limiting
dermatitis characterized by distinctive eruption
manifested as the iris or target lesion.
• TYPES :
a) EM Minor – Localized eruption with no or mild
mucosal involvement
b) EM Major – Mucosal erosions of raised atypical
target lesions
c) STEVEN JOHNSON SYNDROME(SJS) – Mucosal
erosions ; widespread distribution of flat
atypical targets or purpuric macules

13. (i)ETIOLOGY
• Infectious agents :
a.HSV { accounts for 55% of EM major}
b. Mycoplasma infection
c. Subclinical infection of HSV {EM minor}
• Drugs:
Sulfa drugs are most common trigger factors

14. (ii)CLINICAL FEATURES
• Young adults – fourth or fifth decades of life
• Common in males
• Disease is characterized by occurrence of
asymptomatic, vividly erythematous discrete
macules, papules or occasionally vesicles and bullae
distributed in a rather symmetrical pattern most
commonly over the hands and arms, feet and legs ,
face and neck.

15. TARGET LESIONS
• A concentric ring like appearance of the lesions , resulting
from the varying shades of Erythema , occurs in the some
cases and has give rise to TARGET or IRIS or BULL’S EYE
describing them.
• Appears rapidly within a day or two and persist from several
days to a few weeks , gradually fading and eventually clearing

16. ORAL MANIFESTATIONS OF EM
• Pain and discomfort
• Hyperemic macules , papules or vesicles may
become eroded or ulcerated and bleed freely
• Common: Tongue , palate , buccal mucosa and
gingiva

17. STEVEN-JOHNSON SYNDROME
• A severe Bullous form of Erythema
multiforme
• It commences with the abrupt occurrence
of fever, malaise, photophobia and
eruptions of the oral mucosa, genital and
skin
• Patients usually recover unless they
succumb to a secondary infection

18. Oral manifestations SJS
• Severe and painful making mastication impossible
• Mucosal vesicles or bullae occur which ruptures leaving it
covered with a thick white or yellow exudate
• Lips exhibit ulceration with bloody crusting and are painful
• Erosions of pharynx also common
• Mistaken for ANUG

19. • EYE LESIONS:
a. Photophobia
b. Conjunctivitis
c. Panophthalmitis
d. Keratoconjunctivitis sicca
• GENITAL LESIONS:
a. Non specific urethritis
b. Balnitis
c. Vaginal ulcers
• Others:
Tracheo bronchial ulceration and pneumonia

20. HISTOPATHOLOGY
• In general the lesion show intercellular &
intracellular edema of the epithelium with focal
micro vesicle formation.
• Sometimes the edema results in a pooling of an
eosinophilic coagulum within the epithelium.
• A generalized diffuse infiltration of both acute &
chronic inflammatory cells are seen in the
underlying C.T. with vasodilatation of blood
vessels
• C.T. edema & sub-epithelial cleft.

22. TREATMENT
• If drug reaction is suspected ,it should be
withdrawn
• Infections should be treated appropriately after
culture / serologic tests have been performed
• Symptomatic treatment :
1. Oral antihistamines
2. Local skin care
3. Topical steroids
4. Soothing mouth washes
5. Liquid antiseptics { 0.05% chlorhexidine }
• Systemic corticosteroids are controversial

24. • Chronic skin disease characterized by the
appearance of vesicles and bullae , small or
large fluid filled blisters that develop in cycle
• Characterized by intraepithelial bulla
formation
• TYPES:
1. Pemphigus vulgaris
2. Pemphigus foliaceous
3. Paraneoplastic Pemphigus
• Pemphigus vulgaris is the most common
type of oral lesion

25. (i)MECHANISM
• Epithelial cell separation :
Binding of IgG antibody to Pemphigus antigen
leads to the epithelial cell separation by
triggering complement activity . Separation of
cell takes place in lower layer of stratum
spinosum
• Associated factors :
Autoimmune disorders like thymoma,
myasthenia gravis and multiple autoimmune
disorders, it may also be triggered by drug
therapy like penicillamine, penicillin etc

26. (ii)CLINICAL FEATURES
• 5th to 6th decades of life
• Size : thin walled bullae or vesicles varying in
diameter from few mm to several cm on normal skin
• Signs : lesion contain thin , watery fluid shortly after
development , but may become purulent later . They
rapidly break and continue to extend peripherally ,
leaving behind large denuded skin
• Nikolsky sign :
Application of pressure in normal skin >>> New lesion
appears ; reason: upper layer of skin pulling away
from basal layer and prevesicular edema which
disrupts the dermal-epidermal junction.

27. (iii)ORAL MANIFESTATIONS
• Sites: Buccal mucosa { reason : it has less intercellular
substance and fewer intercellular junctions leading to
easier acantholysis} ; palate and gingiva
• Onset: classic bullae on non inflamed base > ruptures
> shallow ulcers
• Symptoms : bleed easily ; severe pain ; unable to eat
• Signs: thin layer of epithelium peels easily in a
irregular pattern leaving behind denuded base

29. (iv)HISTOLOGY
• Intraepithelial vesicle or bullae just above basal
layer producing distinctive suprabasilar split
• Prevesicular edema appears which weakens the
intercellular bridges and junctions
• Loss of cohesiveness {ACANTHOLYSIS} because of
which clumps of epithelium are often found lying
free in the vesicular space { TZANCK CELLS }
• Fluid in the vesicles : variable numbers of PMN
leukocytes and lymphocytes
• SCARCITY of inflammatory cell infiltrate in CT and
vesicular fluid differentiate Pemphigus from other
Bullous

31. TZANCK TEST
• Tzanck test, also Tzanck smear, is scraping of an ulcer
base to look for Tzanck cells.
• Tzanck test is very useful for the diagnosis of PV,
particularly in the early stages of oral Pemphigus
where a biopsy is uncomfortable to the patient
• It reveals multiple acantholytic cells (Tzanck cells). A
typical Tzanck cell is a large round keratinocyte with a
hypertrophic nucleus, hazy or absent nucleoli, and
abundant basophilic cytoplasm.
• The basophilic staining is deeper peripherally on the
cell membrane ("mourning edged" cells) due to the
cytoplasm′s tendency to get condensed at the
periphery, leading to a perinuclear halo.

32. Intercellular staining of epidermal skin cells with fluorescent
markers in a patient with pemphigus vulgaris using direct
immunofluorescence.

33. Pemphigus foliaceous
• Mild form, common in older adults
• Bullous ruptures and DRY to leave masses of flakes or scales
suggestive of an exfoliative dermatitis or eczema
Pemphigus erythematosus
• Senear-usher syndrome
• Bullae or vesicles concomitant with crusted patches ,
ultimate terminate to vulgaris of foliaceous
• Associated fever or malaise
Paraneoplastic Pemphigus
• Neoplasm like lymphoma or chronic leukemia
• Palmar or plantar bullae appear , which does not
occur in other types

34. (v)MANAGEMENT
• Corticosteroids : Topical and systemic
prednisolone
• Combination therapy :
High dose of corticosteroids +
immunosuppressive drugs such as
cyclosporine or azathropine
• Plasmapheresis

36. • Cicatricial pemphigoid (also known as "Benign
mucosal pemphigoid," "Benign mucous membrane
pemphigoid" ,"Ocular pemphigus "and "Scarring
pemphigoid") is a rare chronic autoimmune
sub epithelial blistering disease characterized by
erosive skin lesions of the mucous membranes and skin
that results in scarring of at least some sites of
involvement.
• Etiology : Auto antibodies of IgG subclass , particularly
IgG4 are associated
• Common : Females ; 4th to 5th decades of life ;
• Sites: oral and eye lesion ; also affects skin and genitals
• Feature : Heals by scar

37. •Ocular involvement are the most serious complication
•Adhesions develop between palpebral and bulbar conjunctivae
•Opacity of cornea leads to blindness

39. HISTOPATHOLOGY
• Sub epidermal vesicle or bullae
• No acantholysis
• Non specific Chronic inflammatory infiltrate in connective tissue chiefly
lymphocytes and plasma cells

41. •An chronic, autoimmune ,subepidermal blistering skin
disease that involve mucous membranes.
•The antigens are bullous pemphigoid-antigen 1 (BPAG1;
230kD) which belongs to a family of genes that includes
desmoplakin and bullous pemphigoid antigen 2 (BPAG2;
180kD; Type VII collagen).
•The primary lesion of B.P. is a tense blister which arises
on either normal appearing or erythematous skin
•At times the lesions may arise on urticarial plaques
•Most commonly the lesions are found in the flexural
areas
•Oral lesions are far less frequent

42. ORAL MANIFESTATIONS
• Oral blisters if present, are mild
and transient in contrast to
pemphigus
• Rarely involves oral mucosa
• Gingival lesions resembles
Cicatricial pemphigoid
• Appears extremely
erythematous and may
desquamate
• Also occurs on buccal mucosa,
palate, floor and tongue
• Positive Nikolsky sign

43. HISTOPATHOLOGY:
• The dermal papillae are well preserved but edematous
• Unlike cicatricial, basement membrane remains attached to connective tissue
• No acantholysis
• Vesicles contain a fibrinous exudate admixed with occasional inflamatory cells
• Direct immunofluorescent examination remains the standard way to confirm
the diagnosis

45. •Epidermolysis bullosa ( EB) is a group of inherited bullous
disorders characterized by blister formation in response to
mechanical trauma
•Etiology:
Mutation of genes
• It classified into three types:
1.Epidermolysis bullosa simplex-keratinin genes
2.Junctional Epidermolysis bullosa-laminin genes
3.Dystrophic Epidermolysis bullosa-type VII collagen

46. (ii) Signs and symptoms
•Blistering of the skin
•Tooth decay
• Deformed or lack of finger/toe nails Internal
blistering in the throat, stomach, and intestines
•Scalp blistering and loss of hair
•Excessive sweating
•Hardening of the skin on the feet or hands Thin
skin appearance and white bumps Difficulty
swallowing
•The symptoms vary from the different types.

47. (iii) ORAL MANIFESTATIONS
SOFT TISSUE MANIFESTATIONS:
•Increased fragility
•Perioral lesions
•Microstomia
•Ulcerations
•Ankyloglossia
•Obliteration of oral vestibule
DENTAL MANIFESTATIONS:
• Junctional EB have severe enamel hypoplasia
•Risk for dental caries
•Severe dental crowding and deep bite

49. •Histologic sections of skin show separation of
the epidermis from the dermis in a
subepidermal plane.
•There is absence of a significant inflammatory
response.
• The adjacent epidermis is normal.

51. • Most common mucocutaneous lesion
• Chronic inflammatory disease of the oral mucosa
and skin
• Etiology:
Although the cause is not well known, T cell-
mediated autoimmune phenomena are involved in
the pathogenesis of lichen planus.
• Possible etiology for unmasking the antigen are
contact allergens in restorations or tooth paste.
Lichenoid drug reaction may be due to drugs
• Characteristic feature:
Bilateral white striations, papules, plaques on the
buccal mucosa, tongue and gingiva

52. (i) CLINICAL FEATURES
• Lichen planus affects primarily middle-
aged adults, and the prevalence is
greater among women.
• The classic skin lesions of the cutaneous
form of lichen planus can be described as
six "Ps": pruritic, polygonal,
planar, purple ,papules and
plaques

53. (ii) Signs and symptoms
• The lesions may appear as:
o Lacy, white, raised patches of tissues
o Red, swollen, tender patches of tissues
o Open sores
•Location
oInside of the cheeks, the most common location
oGums
oTongue
oInner tissues of the lips
oThroat
oEsophagus
• Pain or discomfort
The red, inflamed lesions and open sores of oral lichen planus
can cause a burning sensation or pain. The white, lacy patches
alone usually don't cause discomfort, except when they appear
on the tongue.

54. •Other signs or symptoms
oA metallic taste or a blunted taste sensation if the tongue is
affected
oDry mouth
oDifficulty swallowing if the throat or esophagus is affected
oSensitivity to hot or spicy foods
oBleeding and irritation with tooth brushing
Skin. Lesions usually appear as purplish, flat-topped bumps that are
often itchy.
Genitals. Lesions on external genitalia resemble those affecting the
skin. Lesions affecting the mucous membrane of the vagina
resemble those affecting the mouth.
Scalp. When skin lesions appear on the scalp — a rare condition —
they may cause temporary or permanent hair loss.
Nails. Lichen planus of the toenails or fingernails, also rare, may
result in ridges on the nails, thinning or splitting of nails, and
temporary or permanent nail loss.

55. WICKHAM’S STRIAE
•Wickham striae are Characteristic, fine, white or grey
lines or dots seen on the top of the pruritic papular
rash of lichen planus
•The macroscopic appearance of
the histologic phenomenon and hypergranulosis, and
named for Louis Frédéric Wickham.

56. 1.The Reticular Pattern:
•The reticular pattern is considered the
classic form of lichen planus. This form
occurs most frequently, and its white lacy
lines called Wickham's striae characterize
the lesions
• Commonly, the tissue is somewhat
raised and the patient may report that the
areas in contact with the tongue feel
rough in texture

57. 2.The Plaque Form is represented by
thick, plaques of varying sizes, that may
be smooth and appearing somewhat
lighter in color than surrounding tissue
(non-descriptive leukoplakia)
•The tongue is a particular area where
these plaques may be noticed. They often
appear as a "bald" area of concern with
denuded papillae.
•Higher association with malignant
transformation

58. 3.The Papular Pattern :
•The papular pattern is noted by
the small, pin point papules
appearing as small white dots
measuring approximately 0.5 mm
in size.
• The papular pattern may be in
conjunction with other forms of
lichen planus and may be over
looked in an oral examination
depending upon the number of
papules and the distinct
appearance of the lesions

59. 4.The Bullous Form:
•The bullous form consists of vesicles
ranging from a few millimeters in
diameter to much larger vesicles.
•Eating and speaking will usually cause
the vesicles to rupture soon after
forming.
•This form is seen most often on the
buccal mucosa followed by the tongue

60. 5.The Atrophic Form- also called erythematous form.
•The atrophic form presents as diffuse reddened patches that
may be in combination with other forms of lichen planus such as
the white striations of the reticular form.
•Often found on the attached gingiva, the patient may
complain of irritation or burning sensations.
•Lesions are poorly defined and may have striae at the
peripheral areas.
• When this form involves the gingiva, it is sometimes referred
to as "desquamative gingivitis" which is a clinical term used by
some clinicians denoting a reddened appearance of the tissue.
• Other skin diseases may appear similar and this term is not
diagnostic .
• In addition, the atrophic form may be confused with BMMP
and may appear similar to the gingival depicted

61. Atrophic Form consists of red, erythematous Atrophic form sometimes described as
areas and may include a reticular/plaque like desquamative gingivitis. This form when affecting the
surface. gingiva is definitively diagnosed through biopsy and
immunofluorescence.
Red marginal area affected by the lichen
planus and the noted plaque-like areas
superior to the marginal areas.

62. 6.The Ulcerative Form.
•As the term indicates, the ulcerative form features one or
several ulcers that are often seen in association with any of
the other disease forms.
•This form is quite painful for the patient and the soreness
of the tissue will affect eating and brushing of the teeth.
•The ulcerations may affect any tissue surface and may
even extend into the soft tissues of the tonsillar pillars and
esophageals areas.
•This form is reported as having a higher number of
malignant tissue changes.
• The ulcerative nature of this form probably allows more
detrimental tissue changes with chronic irritation and
possibly a port of entry for pathogens.

63. Erosive type of lichen planus with loss of A mixture of erosive and reticular lichen
surface epithelium in the buccal mucosa. planus with some candida albicans present
on tongue and buccal mucosa.
Highly erosive area of lichen planus in
the soft plate region

64. Lichenoid Reactions:
•The most common material used in dentistry that may cause a
lichenoid reaction is amalgam, although other known dental materials
have been implicated as well. The mercury in the metal alloy usually
causes the hypersensitivity reactions
•Hypersensitivity reactions may also be due to flavoring agents or food
products or drugs
• The lesions are usually isolated and they do not subside as long as the
tissue is in contact with the restoration

65. (iii) HISTOPATHOLOGY
•The epidermis is hyperkeratotic with irregular acanthosis and focal
thickening in the granular layer.(wedge-shaped hypergranulosis)
•The upper dermis has a band like infiltrate of lymphocytic
(primarily helper T) and histiocytic cells with many Langerhans cells
at the dermal-epidermal junction.
•The lymphocytes are intimately associated with basal keratinocytes
, which shows degeneration ,necrosis & resemblance in size &
contour to more mature cells of Stratum spinosum. This is
SQUAMATIZATION.
•As a consequence to this destructive infiltration ,results in redifining
of normal smoothly undulating configuration of dermoepidermal
junction to more angulated zigzag contour ("saw-tooth" appearance
of the rete pegs).
•Pigment incontinence due to damage of basal keratinocytes and
melanocytes.(this leads to hyperpigmentation of lesion)

66. Saw tooth rete pegs

67. CIVATTE BODIES:
•Anucleate ,necrotic basal cells become incorporated into inflamed papillary
dermis -these are civatte bodies or colloid bodies.
•These are characteristic of lichen planus (but can be found in any chronic
dermatitis where basal keratinocytes are injured)
•In addition to apoptotic keratinocytes, colloid bodies are composed of globular
deposits of IgM(occasionally immunoglobulin G [IgG] or immunoglobulin A [IgA])
and complement.
•Max Joseph clefts : Basal keratinocytes weakens the epithelial interface causing
histologic clefts

68. (iv) MANAGEMENT
1.Corticosteroids 3.Nonsteroidal ointments
Corticosteroids may reduce •Calcineurin inhibitors, which are
inflammation associated with oral closely related to or identical to oral
lichen planus. The side effects vary medications used to prevent rejection
depending on types of usage of transplanted organs.
•Topical. mouthwash or ointment •These treatments appear to be
•Oral. taken as a pill effective for the treatment of oral lichen
•Injections. Injections may be planus. Examples :Tacrolimus (Protopic
administered directly into lesions. ointment) and pimecrolimus (Elidel
Repeated use of corticosteroid cream).
injections can cause some of the same
side effects as oral corticosteroids. 4.Addressing triggers
Drugs. stop the drug or try an alternative
drug
2.Retinoids Allergen. advised to avoid the allergen
Retinoid are synthetic versions of vitamin Stress. stress may be a factor that
that can be applied as a topical ointment complicates symptoms or triggers the
or taken orally. recurrence

70. • White sponge nevus (WSN) is a rare autosomal
dominant disorder, with a high degree of
penetrance and variable expressivity.
• The cause is a mutation in the mucosal keratin 4
or keratin 13 genes. Since it is inherited, it can
be present at birth or at puberty.
• It is a benign, uncommon, and predominantly
affects non-keratinized stratified-squamous
epithelia

71. (i) ORAL MANIFESTATIONS
•A thick, corrugated bilateral white plaque with a
spongy texture, usually on the buccal mucosa, but
sometimes on the labial mucosa, alveolar ridge or
floor of the mouth.
•Asymptomatic
•The gingival margin and dorsum of the tongue are
almost never affected.
•Although this condition is perfectly benign, it is
often mistaken for leukoplakia
•Ragged white areas which can be removed by
gentle rubbing

73. (ii) HISTOPATHOLOGY
•Hyperparakeratosis epithelium , acanthosis
present
•Basal layer intact
•Cells of spinous layer show Intracellular
edema
•Submucous have inflammatory cell
infiltrate
•Vacuolated and dyskeratotic epithelial
keratinocytes are present that demonstrate
perinuclear eosinophilic condensations.

74. cells of spinous layer show
a shrunken nucleus (fried
egg appearance)
Hyperparakeratosis,
acanthosis, and spongiosis
in stratified squamous
epithelium. The associated
connective tissue is usually
free of inflammation