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DESQUAMATIVE GINGIVITIS
Presented By UTPAL HALDER ( INTERN )
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
Etiology
. Certain dermatoses
. Hormonal influences
.Abnormal responses to the irritation
. Chronic infections
. Idiopathic
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
. Erythema
Mild form :
. Painless
.Age 17 - 23 years
. Common in females
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 .
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 .
Systemic approach to diagnosis of
Desquamative gingivitis
. Clinical History .
. Clinical Examination .
. Biopsy .
. Microscopic Examination
. Immunoflouroscence .
1. Direct & Indirect
Diseases clinically presenting
as Desquamative Gingivitis
ORAL LICHEN PLANUS
Chronic inflammatory disease that affects skin
and mucous membrane .
. Wilson - 1869
Epidemiology
. Prevalence : 1.5%
. Women > Men
. Predominant in adults > 40 years
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 .
Clinical features
The skin
lesion of
OLP appears
as small ,
angular , flat
topped
papule .
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 .
RETICULAR :
Wickham’s striae
Plaque like Lichen Planus :
Slightly raised or flat
white area on the oral
mucous membrane .
Plaque type lesions may
clinically similar to
Homogenous
Leukoplakia .
Erosive Lesions :
These extensive
erythematous areas with
a patchy distribution
may present as focal or
diffuse hemorrhagic
areas .
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 .
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 .
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 .
Differential Diagnosis :
. Lupus erythematous
. Lichenoid reaction
. Cicatrical pemphigoid
. Chronic ulcerative stomatitis
. Pemphigus vulgaris
. Leukoplakia
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 .
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 .
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%
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 .
CLINICAL FEATURES :
. EXTRAORAL FEATURES :
. Nasopharyngeal involvement is characterised by rupture
of vesicles in nasal mucosa .
. Dysphagia .
. Dyspnea and laryngeal stenosis .
Occular Involvement :
. Trichiasis
. Entropion .
.Ankyloblepharon .
. Symblepharon .
ORAL MANIFESTATIONS :
Vesiculo-bullous
lesions are seen
in gingiva .
Severe erythema
may remain for
months .
HISTOPATHOLOGY :
. Chronic inflammatory
infiltrate .
. Separation of epithelium
and connective tissue
. Sub epithelial vesiculation
IMMUNOFLUORESCENCE :
Positive
immunofluoresce
nce . The main
immunoreactants
are IgG & C3 .
DIFFERENTIAL DIGNOSIS :
1. Bullous pemphigoid .
2. Bullous Lichen planus .
3. Pemphigus vulgaris .
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
BULLOUS PEMPHIGOID :
Chronic ,
autoimmune , sub
epidermal
blistering skin
disease that rarely
involves mucous
membrane .
ORAL LESIONS :
. Gingiva : Erythematous and
Desquamate
. Painful.
. Nikolsky’s sign : Negative
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 .
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 .
CLINICAL FEATURES :
1. Bullae .
2. Erosion .
3. Ulcers .
4. Nikolsky test is positive .
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 .
IMMUNOFLUORESCENCE :
Direct immunofluorescence
of oral pemphigus . Positive
intercellular signal for
immunoglobulin G ( IgG )
deposits is seen in
keratinocytes of the
stratified squamous
epithelium .
DIFFERENTIAL DIAGNOSIS :
1. Pemphigoid .
2. Lichen planus .
3. Linear IgA disease .
4. Chronic ulcerative stomatitis .
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 .
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 .
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 .
IMMUNOFLUORESCENCE :
Direct
Immunofluorescence of
chronic ulcerative
stomatitis . Nuclear
deposits of IgG are
prominent in the basal
cell layer and fade
toward the superficial
layers .
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 .
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 .
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 .
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 .
TREATMENT :
. Combination of Dapsone and Sulfones .
. Small amount of Prednisone ( 10 - 30 mg/
day) can be added if the initial response is
inadequate .
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 .
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 .
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 .
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 .
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 .
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 .
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 .
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 .
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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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 .
  • 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 .