This document provides information on various conditions that can present as desquamative gingivitis, including their signs, symptoms, pathogenesis, histopathology and treatment. Oral lichen planus, pemphigoid, pemphigus vulgaris, chronic ulcerative stomatitis, linear IgA disease and lupus erythematosus are discussed in detail. The document also covers erythema multiforme and provides an overview of the differential diagnosis and evaluation of desquamative gingivitis.

1. DESQUAMATIVE GINGIVITIS
Presented By UTPAL HALDER ( INTERN )

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

3. Etiology
. Certain dermatoses
. Hormonal influences
.Abnormal responses to the irritation
. Chronic infections
. Idiopathic

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

5. . Erythema
Mild form :
. Painless
.Age 17 - 23 years
. Common in females

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

10. Epidemiology
. Prevalence : 1.5%
. Women > Men
. Predominant in adults > 40 years

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 .

12. Clinical features
The skin
lesion of
OLP appears
as small ,
angular , flat
topped
papule .

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 .

14. RETICULAR :
Wickham’s striae

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 .

20. Differential Diagnosis :
. Lupus erythematous
. Lichenoid reaction
. Cicatrical pemphigoid
. Chronic ulcerative stomatitis
. Pemphigus vulgaris
. Leukoplakia

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 .

26. Occular Involvement :
. Trichiasis
. Entropion .
.Ankyloblepharon .
. Symblepharon .

27. ORAL MANIFESTATIONS :
Vesiculo-bullous
lesions are seen
in gingiva .
Severe erythema
may remain for
months .

28. HISTOPATHOLOGY :
. Chronic inflammatory
infiltrate .
. Separation of epithelium
and connective tissue
. Sub epithelial vesiculation

29. IMMUNOFLUORESCENCE :
Positive
immunofluoresce
nce . The main
immunoreactants
are IgG & C3 .

30. DIFFERENTIAL DIGNOSIS :
1. Bullous pemphigoid .
2. Bullous Lichen planus .
3. Pemphigus vulgaris .

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 .

33. ORAL LESIONS :
. Gingiva : Erythematous and
Desquamate
. Painful.
. Nikolsky’s sign : Negative

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 .

36. CLINICAL FEATURES :
1. Bullae .
2. Erosion .
3. Ulcers .
4. Nikolsky test is positive .

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 .

39. DIFFERENTIAL DIAGNOSIS :
1. Pemphigoid .
2. Lichen planus .
3. Linear IgA disease .
4. Chronic ulcerative stomatitis .

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 .

43. IMMUNOFLUORESCENCE :
Direct
Immunofluorescence of
chronic ulcerative
stomatitis . Nuclear
deposits of IgG are
prominent in the basal
cell layer and fade
toward the superficial
layers .

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 .

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