2. Differential diagnosis is not easy as all oral mucosa
lesion are the same , with short lived bullous vesicles
which burst , causing ulcerations . Hence the name
desquamative gingivitis
4. Chronic Desquamative Gingivitis
. Chronic desquamative gingivitis is characterised by intense
redness and desquamative of the surface epithelium the
attached gingiva .
Clinical features of desquamative gingivitis vary in severity
. Mild form
. Moderate form
. Severe form
6. Moderate form :
. Patchy distribution of bright red and grey areas .
. Surface is smooth and shiny and soft in consistency .
. Slight pitting on pressure
. Nicolsky’s sign positive
. Remainder of the mucosa
is also extremely Smooth
and shiny
.Age : 30 - 40 years .
. ℅ of burning sensation and
sensitivity to the thermal
changes .
7. Severe form :
. Scattered , irregularly shaped
areas - striking red appearance.
.Areas is greyish blue giving an
overall speckled appearance .
. Surface epithelium - shredded
and friable and can be peeled off
in small patches .
. Patient cannot tolerate coarse
food , condiments and
temperature changes .
. Constant dry and burning sensation
throughout the oral cavity .
8. Systemic approach to diagnosis of
Desquamative gingivitis
. Clinical History .
. Clinical Examination .
. Biopsy .
. Microscopic Examination
. Immunoflouroscence .
1. Direct & Indirect
9. Diseases clinically presenting
as Desquamative Gingivitis
ORAL LICHEN PLANUS
Chronic inflammatory disease that affects skin
and mucous membrane .
. Wilson - 1869
11. Pathogenesis :
. Current data suggest that OLP is a T cell -
mediated autoimmune disease in which
auto - cytotoxic CD8+ T cell trigger
apoptosis of oral epithelial cells .
. However , the precise cause of OLP is
unknown .
13. ORAL LESIONS :
. Oral lichen planus presents as white striations ,
white papule , white plaques , erythema , erosions
or blisters .
. Presents in a variety of forms :
1. Reticular .
2.Atrophic .
3. Papular .
4. Ulcerative
5. Bullous Forms .
15. Plaque like Lichen Planus :
Slightly raised or flat
white area on the oral
mucous membrane .
Plaque type lesions may
clinically similar to
Homogenous
Leukoplakia .
16. Erosive Lesions :
These extensive
erythematous areas with
a patchy distribution
may present as focal or
diffuse hemorrhagic
areas .
17. Vesicular or Bullous lesions :
These lesions are raised , fluid - filled and are
uncommon . Short lived on the gingiva ,
quickly rupturing and leaving an ulceration.
Atrophic lesions :
Atrophy of the gingival
tissues with ensuing
epithelial thinning
results in erythema
coffined to the gingiva .
18. Histopathology :
Biopsy from the gingival
lesions shows
hyperkeratosis and mild
hypergranulosis . Focal
basal cell degeneration,
lymphocytic exocytosis
and thickening of
basement membrane are
apparent . The rate pegs
exhibited a slight serrated
configuration .
19. IMMUNOPATHOLOGY
DI - Linear fibrillar
deposits of fibrin in the
basement membrane
zone . Scattered
immunoglobulin -
staining cytoid bodies
in the upper areas of
the lamina propria .
. Serum tests using indirect
Immunofluorescence are
negative in lichen planus .
21. TREATMENT :
. The keratotic lesions of oral lichen planus are
asymptomatic and do not require treatment .
. The erosive , bullous , or ulcerative lesions of oral lichen
planus are treated with high-potency topical steroid such
as 0.05% fluocinonide ointment ( three times daily ) .
. It can also be mixed 1:1 with carboxymethyl cellulose
( Orabase ) paste or other adhesive ointment .
. SEVERE CASES - Intralesional injections of
triamcinolone acetonide ( 10-20 mg ) or short term
regimens of 40 mg prednisone daily for 5 days followed by
10 to 20 mg daily for an additional 2 weeks .
22. PEMPHIGOID :
2. Cicatrical / mucous membrane pemphigoid
. Types of pemphigoid that are as follows :
1. Bullous pemphigoid .
3.Antiepiligrin pemphigoid
. Hippocrates was first to describe pemphigoid as a type
of fever accompanied by blisters .
23. MUCOUS MEMBRANE
PEMPHIGOID :
Cicatrical pemphigoid :
. Chronic , vesiculobullous , autoimmune disorder
. It predominantly affects women in fifth decade of life .
. The percentage of involvement is :
. Oral mucosal bulls lesion : 85-90%
. Occular lesions : 66%
. Nasal lesions : 15-23%
. Laryngeal involvement : 8- 12%
24. PATHOGENESIS :
. The two major antigenic determinants for cicatrical
pemphigoid are bullous pemphigoid 1& 2 ( BP1 &BP2 )
. Most cases of Cicatrical pemphigoid are the result of an
immune response directed against BP2 and less commonly
against BP1 and epiligrin .
25. CLINICAL FEATURES :
. EXTRAORAL FEATURES :
. Nasopharyngeal involvement is characterised by rupture
of vesicles in nasal mucosa .
. Dysphagia .
. Dyspnea and laryngeal stenosis .
31. TREATMENT :
. Localized lesions : Fluocinonide ( 0.05% ) and clobetasol
propionate ( 0.05% ) in an adhesive vehicle can be used
three times a day for up to 6 months .
. If occultar involvement exists , systemic corticosteroids
are indicated .
. When. Lesions do not respond to steroids , systemic
Dapsone ( 4-4 diaminodiphenylsulfone ) has proven to be
effective .
SEVERE CASES : Intravenous immunoglobulins are
another effective but expensive treatment option in high-
risk patients
32. BULLOUS PEMPHIGOID :
Chronic ,
autoimmune , sub
epidermal
blistering skin
disease that rarely
involves mucous
membrane .
34. HISTOPATHOLOGY :
. No evidence of acantholysis .
. Developing vesicles are sub
epithelial rather than
intraepithelial .
. The epithelium separates from
the underlying connective tissue
at the basement membrane zone .
35. PEMPHIGUS VULGARIS :
. Derived from Greek word pemphix ( bubble or blister )
. Pemphigus vulgaris is
most common of
pemphigus diseases, which
also includes
1. P. foliaceous .
2. P. vegetens .
3. P. erythematous .
. Pathogenesis : Circulating autoantibodies
are responsible for disruption of Intercellular
junctions and loss of cell to cell adhesion .
37. HISTOPATHOLOGY :
Typical intraepithelial
clefting with
‘Tombstone’ appearance of
basal cells , which remain
attached to Subjacent
basement membrane and
fibrous connective tissue .
Acantholysis of epithelial
cells with formation of “
Tzanck cells “ is seen in the
intraepithelial cleft .
38. IMMUNOFLUORESCENCE :
Direct immunofluorescence
of oral pemphigus . Positive
intercellular signal for
immunoglobulin G ( IgG )
deposits is seen in
keratinocytes of the
stratified squamous
epithelium .
40. CHRONIC ULCERATIVE
STOMATITIS :
. Condition presents with chronic oral ulcerations
. Predilection for women ( 4th decade )
. Erosions and ulcerations in oral cavity - few cases
with cutaneous lesions .
41. ORAL LESIONS :
Painful , solitary , small
blisters and erosions with
surrounding erythema -
mainly on gingiva and
lateral border of the
tongue : hard palate may
also present similar
lesions .
42. HISTOPATHOLOGY :
. Hyperkeratosis , acanthosis , and liquefaction of the
basal layer areas of sub epithelial clefting .
. Underlyng lamina propia - lumphohistiocytic
chronic infiltrate in a band like configuration .
44. DIAGNOSIS :
Direct and indirect immunofluorescence required to
arrive at correct diagnosis .
TREATMENT :
Mild cases : Topical steroids ( flucononide , clobetasol
propionate ) and topical tetracycline .
Severe cases : Systemic steroids .
Hydroxychloroquinine sulphate 200-400 mg/day .
45. LINEAR IgA DISEASE :
Uncommon mucocutaneous disorder with
predilection in women .
CLINICAL FEATURES :
Pruritic vesiculobullous rash during middle to late age .
Plaques or crops with an annular presentation surrounded
by a peripheral rim of blisters .
Skin of upper and lower trunk , shoulders , groin and
lower limbs - face and perineum may also be affected .
46. ORAL LESIONS :
Mucosal - oral involvement - 50-100% of cases
. Vesicles .
. Erosive gingivitis / chelitis .
. Hard and soft palate
commonly affected - tonsillar
pillars , buccal mucosa , tongue
and gingiva .
. Painfull ulcerations or erosions
. Occasionally oral lesion only
manifestation for several years
before cutaneous lesions .
47. IMMUNOFLUORESCENCE :
Linear deposits of IgA are observed at the epithelial tissue-
connective tissue interface .
Differential Diagnosis :
1. Erosive lichen planus .
2. Chronic ulcerative stomatitis .
3. Pemphigus vulgaris .
4. Bullous pemphigoid .
5. Lupus erythematosus .
48. TREATMENT :
. Combination of Dapsone and Sulfones .
. Small amount of Prednisone ( 10 - 30 mg/
day) can be added if the initial response is
inadequate .
49. LUPUS ERYTHEMATOSUS :
. It is an autoimmune disease with three different
clinical presentations .
. 1. Systemic Lupus Erythematosus .
. 2 . Chronic Cutaneous Lupus Erythematosus .
. 3 . Subacute Cutaneous Lupus Erythematosus .
50. SYSTEMIC LUPUS ERYTHEMATOSUS :
1. Females : Males - 10:1
2.Affects kidneys , skin and
mucosa .
3. Fever , weight loss and
arthritis .
4. Rash on malar area .
5. Oral lesions are present in
up to 40% of patients .
51. CHRONIC CUTANEOUS
LUPUS ERYTHEMATOSUS :
Chronic cutaneous lupus
erythematosus . Multiple
facial lesions with
irregular hyperpigmented
borders , some of which
exhibit central scarring
with cutaneous atrophy .
Other lesions consists of
hyperpigmented cutaneous
patches .
52. IMMUNOFLUORESCENCE :
Direct immunofluorescence of the lesional tissue reveals
immunoglobulins and C3 deposits at the dermal -
epidermal junction of the lesional and perilesional
tissue but not in the normal tissue .
TREATMENT :
. Topical and intralesional corticosteroids .
. Systemic corticosteroids alone or in combination with
other Immunosuppressive agents such as
cyclophosphamide .Antimalarial drugs may topical or
systemic retinoids may be beneficial .
. Gold salts and cyclosporin .
53. ERYTHEMA MULTIFORME :
.An acute bullous and or macular
inflammatory mucocutaneous disease where a
series of immunopathologic mechanisms occur .
CLINICAL FEATURES :
1. Herpes simplex infections .
2. Mycoplasma infection .
3. Drug reactions : sulphonamides , penicillin’s ,
phenylbutazone and phenytoin .
4. Hemorrhagic crusting of the vermillion
border of lips common .
54. 5 . Presence of crusting Important in arriving at diagnosis .
8. Lesions - so painful that chewing and swallowing is
impaired
6. Target or iris lesions with central clearing
9. Erythema multiforme minor - lasts approx 4 weeks .
11. Stevens-Johnson syndrome - lasts month or longer .
Involves skin , conjunctiva , oral mucosa and genitalia
requiring more aggressive therapy .
7. Multiple , large , shallow , painful ulcers with an
erythematous birders .
10. Moderate cutaneous and mucosal involvement .
55. ERYTHEMA MULTIFORME :
Large , shallow and
painful ulcers
involving the labial
and buccal mucosa .
Hemorrhagic crusting
of the mandibular
vermilion border of
the lips is observed .
56. HISTOPATHOLOGY :
1. Liquefaction degeneration of upper epithelium and
intraepithelial micro-vesicles but without acantholysis .
2. Pseudoepitheliomatous hyperplasia and nearotic
keratinocytes .
3. Degenerative changes in the basement membrane .
4. Dense inflammatory cell infiltrate at the junction of
epithelium and lamina propria , which becomes indistinct .
5. Edema of the lamina propria . Vascular dilation and
congestion are also present .
57. IMMUNOFLUORESCENCE :
Negative in Erythema Multiforme .
TREATMENT :
. No specific treatment for Erythema Multiforme .
. For mild symptoms , systemic and local anti histamines
together with topical anesthetics and debridement of the
lesions with an oxygenating agent are required .
. In the patients with bullous or ulcerative lesions and severe
symptoms , corticosteroids are considered the drug of choice .