Vesiculo-Bollous Diseases 2
Aiman A. Ali, DDS, PhD.
Associate Professor, Oral Pathology & Medicine
College of Dentistry, K...
Vesiculo-Bollous Diseases
Viral
 Associated with immunologic
defects
 Hereditary


Aiman A. Ali DDS, PhD.
Vesiculo-Bollous Diseases 2
Associated with immunologic defects

Pemphigus Vulgaris
Cicatricial pemphigoid
Bullous pemphig...
Pemphigus
Vulgaris
Aiman A. Ali DDS, PhD.
Etiology
 Reactive

IgG against epithelial desmosometonofilament complexes
 Loss of cell-to-cell adherence (acantholysis...
Clinically
 Mucocutaneuos

disease
 Skin lesions appear after OL in a period of 1
year
 Ulcers preceded by bullae
 60%...
Aiman A. Ali DDS, PhD.
Clinically

Aiman A. Ali DDS, PhD.
Histopathologically
 Acantholysis
 Tzanck

cells [free-floating rounded or
spherical SSC]

 Basal

layer remains attach...
Aiman A. Ali DDS, PhD.
Tzanck cells

Aiman A. Ali DDS, PhD.
Aiman A. Ali DDS, PhD.
Aiman A. Ali DDS, PhD.
Immunofluorescence
Direct

Aiman A. Ali DDS, PhD.

Indirect
Indirect Immunofluorescence

Appear

in 80% of Pemphigus Vulgaris
patients

To

assess the severity of the lesion

Aiman...
Aiman A. Ali DDS, PhD.
Aiman A. Ali DDS, PhD.
Aiman A. Ali DDS, PhD.
Differential diagnosis
 Pemphigoid
 Erythema

(bullous or cicatricial)

multiform

 Bullous lichen

planus

 Dermatiti...
Pemphigus vegetans
 Skin,

vermilion and oral mucosa
 Histopathologically: epithelial hyperplasia
with intraepithelial a...
Aiman A. Ali DDS, PhD.
Treatment
 High

dose of corticosteroids
 Immunosuppressant agents to reduce
complications of SAIDs as (osteoporosis,
hy...
Paraneoplastic Pemphigus
 Simulate

pemphigus vulgaris clinically
 Associated with lymphoma or other
malignancies
 Hist...
Cicatricial
Pemphigoid
Aiman A. Ali DDS, PhD.
Etiology
 Benign

mucous membrane pemphigoid,
ocular pemphigus, childhood pemphigoid,
and mucosal pemphigoid

 Idiopathi...
{

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{

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Clinical features
 More

common among adult women

 Chronic

lesions appear as vesiculo-bullous eruptions
involve oral m...
Histopathology
 Sub-basal clefting

with clear cut
separation at the basement membrane
 No evidence of acantholysis
 Va...
Aiman A. Ali DDS, PhD.
Aiman A. Ali DDS, PhD.
Immunofluorescence
 Direct IF

of intact oral mucosa demonstrate
linear pattern of IgG fluorescence
 Occasionally IgA ma...
Aiman A. Ali DDS, PhD.
Aiman A. Ali DDS, PhD.
Aiman A. Ali DDS, PhD.
Differential diagnosis
Pemphigus

vulgaris
Erosive lichen planus

Aiman A. Ali DDS, PhD.
Aiman A. Ali DDS, PhD.
Treatment
 Topical

corticosteroids (betamethasone
dexamethasone…etc)
 In severe cases systemic SAIDs with
immunosuppres...
Aiman A. Ali DDS, PhD.
Bullous
pemphigoid
Aiman A. Ali DDS, PhD.
Etiology
 Similar

to cicatricial pemphigoid

 There

are circulating autoantibodies to
basement membrane zone antigen

...
Clinical features

 Very

common in the 7th and 8th decades

 Lesions

affect the skin
Histopathology
 Normal HP

the same of CP

 Ultrastructurally:

the basement membrane

is cleaved at the level of lamina...
Immunopathology
 There

is a detectable level of circulating

antibodies in 70% of cases
 However,

no correlation with ...
Treatment

 Systemic

corticosteroids
Dermatitis
herpetiformis
Etiology
 Unknown

cause

 Deposits of
 No

IgA in the skin and mucosa

circulating autoantibodies in the

patient’s se...
Clinical features


Chronic disease typically seen in young adults



Cutaneous disease, rarely appear in the oral cavit...
Histopathology
 Accumulation

of neutrophils and eosinophils
producing dermal micro-abscess

 Connective

tissue become ...
Immunopathology
 Immunofluorescent

staining is positive
at the epidermal-dermal junction

 Almost IgA

alone or in comb...
Treatment

 It dose

not respond to SAIDs

 Sulfapyridine is the
 Gluten-free

diet

treatment of choice
Linear IgA
Disease
 IgA deposits at

the dermal-epidermal
junction in linear pattern

 Not

associated with gluten-sensitive
enteropathy

...
Epidermolysis
Bullosa
Etiology



Hereditary disease



In one type acquired



Formation of blisters at sites of minor trauma
Clinical features
 Muco-Cutaneous disease
 It has

several different forms:
 EB Simplex
 EB dystrophic dominant
 EB d...
Treatment
Symptomatic
{

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Vesiculo bullous II
Vesiculo bullous II
Vesiculo bullous II
Vesiculo bullous II
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Vesiculo bullous II

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Oral Pathology I
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Vesiculo bullous II

  1. 1. Vesiculo-Bollous Diseases 2 Aiman A. Ali, DDS, PhD. Associate Professor, Oral Pathology & Medicine College of Dentistry, King Faisal University
  2. 2. Vesiculo-Bollous Diseases Viral  Associated with immunologic defects  Hereditary  Aiman A. Ali DDS, PhD.
  3. 3. Vesiculo-Bollous Diseases 2 Associated with immunologic defects Pemphigus Vulgaris Cicatricial pemphigoid Bullous pemphigoid Dermatitis herpetiformis Linear IgA Disease Aiman A. Ali DDS, PhD. Hereditary Epidermolysis Bullosa
  4. 4. Pemphigus Vulgaris Aiman A. Ali DDS, PhD.
  5. 5. Etiology  Reactive IgG against epithelial desmosometonofilament complexes  Loss of cell-to-cell adherence (acantholysis) Pemphigus antibody + Target antigen Activate epithelial intracellular protolytic enzyme Desmosome-tonofilament complex Aiman A. Ali DDS, PhD. Acantholysis
  6. 6. Clinically  Mucocutaneuos disease  Skin lesions appear after OL in a period of 1 year  Ulcers preceded by bullae  60% of cases the first appearance in the oral cavity  More common in the 4th and 5th decade  Nikolsky sign is positive Aiman A. Ali DDS, PhD.
  7. 7. Aiman A. Ali DDS, PhD.
  8. 8. Clinically Aiman A. Ali DDS, PhD.
  9. 9. Histopathologically  Acantholysis  Tzanck cells [free-floating rounded or spherical SSC]  Basal layer remains attached to the basement membrane  Bulla or vesicle are filled with fluid, Tzanck cells and neutrophils Aiman A. Ali DDS, PhD.
  10. 10. Aiman A. Ali DDS, PhD.
  11. 11. Tzanck cells Aiman A. Ali DDS, PhD.
  12. 12. Aiman A. Ali DDS, PhD.
  13. 13. Aiman A. Ali DDS, PhD.
  14. 14. Immunofluorescence Direct Aiman A. Ali DDS, PhD. Indirect
  15. 15. Indirect Immunofluorescence Appear in 80% of Pemphigus Vulgaris patients To assess the severity of the lesion Aiman A. Ali DDS, PhD.
  16. 16. Aiman A. Ali DDS, PhD.
  17. 17. Aiman A. Ali DDS, PhD.
  18. 18. Aiman A. Ali DDS, PhD.
  19. 19. Differential diagnosis  Pemphigoid  Erythema (bullous or cicatricial) multiform  Bullous lichen planus  Dermatitis herpetiformis  Paraneoplastic pemphigus  In syndrome small lesions, aphthous stomatitis Aiman A. Ali DDS, PhD.
  20. 20. Pemphigus vegetans  Skin, vermilion and oral mucosa  Histopathologically: epithelial hyperplasia with intraepithelial abscess formation  Abundant eosinophils Aiman A. Ali DDS, PhD.
  21. 21. Aiman A. Ali DDS, PhD.
  22. 22. Treatment  High dose of corticosteroids  Immunosuppressant agents to reduce complications of SAIDs as (osteoporosis, hyperglycemia, hypertension)  When SAIDs are contraindicated Gold therapy is recommended Aiman A. Ali DDS, PhD.
  23. 23. Paraneoplastic Pemphigus  Simulate pemphigus vulgaris clinically  Associated with lymphoma or other malignancies  Histopathologically and IF is different Aiman A. Ali DDS, PhD.
  24. 24. Cicatricial Pemphigoid Aiman A. Ali DDS, PhD.
  25. 25. Etiology  Benign mucous membrane pemphigoid, ocular pemphigus, childhood pemphigoid, and mucosal pemphigoid  Idiopathic autoimmune disease  Deposit of IG and complement components along the basement zone  Usually no circulating antibodies Aiman A. Ali DDS, PhD.
  26. 26. { { { { { {
  27. 27. Clinical features  More common among adult women  Chronic lesions appear as vesiculo-bullous eruptions involve oral mucosa, which heal with scaring  When affects gingiva exclusively is referred to as gingivosis or desquamative gingivitis  Other sites: conjunctiva, larynx, genitalia, and esophagus  Skin lesions are uncommon  Nikolsky’s sign is positive Aiman A. Ali DDS, PhD.
  28. 28. Histopathology  Sub-basal clefting with clear cut separation at the basement membrane  No evidence of acantholysis  Variable infiltration with lymphocytes, plasma cells and occasionally eosinoand neutrophils  Blood vessels often are dilated Aiman A. Ali DDS, PhD.
  29. 29. Aiman A. Ali DDS, PhD.
  30. 30. Aiman A. Ali DDS, PhD.
  31. 31. Immunofluorescence  Direct IF of intact oral mucosa demonstrate linear pattern of IgG fluorescence  Occasionally IgA may detected  Complement components are commonly found  Indirect IF studies are usually negative Aiman A. Ali DDS, PhD.
  32. 32. Aiman A. Ali DDS, PhD.
  33. 33. Aiman A. Ali DDS, PhD.
  34. 34. Aiman A. Ali DDS, PhD.
  35. 35. Differential diagnosis Pemphigus vulgaris Erosive lichen planus Aiman A. Ali DDS, PhD.
  36. 36. Aiman A. Ali DDS, PhD.
  37. 37. Treatment  Topical corticosteroids (betamethasone dexamethasone…etc)  In severe cases systemic SAIDs with immunosuppressive agents Aiman A. Ali DDS, PhD.
  38. 38. Aiman A. Ali DDS, PhD.
  39. 39. Bullous pemphigoid Aiman A. Ali DDS, PhD.
  40. 40. Etiology  Similar to cicatricial pemphigoid  There are circulating autoantibodies to basement membrane zone antigen  Degeneration of basement membrane attachment complexes  Separation occur at the lamina lucida plane
  41. 41. Clinical features  Very common in the 7th and 8th decades  Lesions affect the skin
  42. 42. Histopathology  Normal HP the same of CP  Ultrastructurally: the basement membrane is cleaved at the level of lamina lucida
  43. 43. Immunopathology  There is a detectable level of circulating antibodies in 70% of cases  However, no correlation with the level of clinical disease  IF findings corresponding to those in CP
  44. 44. Treatment  Systemic corticosteroids
  45. 45. Dermatitis herpetiformis
  46. 46. Etiology  Unknown cause  Deposits of  No IgA in the skin and mucosa circulating autoantibodies in the patient’s serum
  47. 47. Clinical features  Chronic disease typically seen in young adults  Cutaneous disease, rarely appear in the oral cavity  Symmetrical aggregated vesicular lesions of the skin with face and scalp involvement  Periods of exacerbation and remission  Iodide component exacerbate some cases  Orally lesions appear as superficial ulcers with fibrinous base preceded by vesicles
  48. 48. Histopathology  Accumulation of neutrophils and eosinophils producing dermal micro-abscess  Connective tissue become necrotic and the overlying epithelium separate  Formation of subepithelial vesicle
  49. 49. Immunopathology  Immunofluorescent staining is positive at the epidermal-dermal junction  Almost IgA alone or in combination with IgG or IgM
  50. 50. Treatment  It dose not respond to SAIDs  Sulfapyridine is the  Gluten-free diet treatment of choice
  51. 51. Linear IgA Disease
  52. 52.  IgA deposits at the dermal-epidermal junction in linear pattern  Not associated with gluten-sensitive enteropathy  Common oral lesions  Separation at the basement membrane
  53. 53. Epidermolysis Bullosa
  54. 54. Etiology  Hereditary disease  In one type acquired  Formation of blisters at sites of minor trauma
  55. 55. Clinical features  Muco-Cutaneous disease  It has several different forms:  EB Simplex  EB dystrophic dominant  EB dystrophic recessive (Oral Manifestations)  Junctional EB  EB Acquista  Very common in • Hereditary (newborns and early childhood) • Acquired (adulthood)
  56. 56. Treatment Symptomatic
  57. 57. { { { { { {

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