This document discusses several vesiculo-bullous diseases including pemphigus vulgaris, cicatricial pemphigoid, bullous pemphigoid, dermatitis herpetiformis, linear IgA disease, and epidermolysis bullosa. It provides information on the etiology, clinical features, histopathology, immunofluorescence findings, differential diagnosis, and treatment for each condition. The document is authored by Aiman A. Ali, an associate professor of oral pathology and medicine at King Faisal University.

1. Vesiculo-Bollous Diseases 2
Aiman A. Ali, DDS, PhD.
Associate Professor, Oral Pathology & Medicine
College of Dentistry, King Faisal University

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

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. Pemphigus
Vulgaris
Aiman A. Ali DDS, PhD.

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. 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.

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8. Clinically
Aiman A. Ali DDS, PhD.

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.

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11. Tzanck cells
Aiman A. Ali DDS, PhD.

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

15. Indirect Immunofluorescence
Appear
in 80% of Pemphigus Vulgaris
patients
To
assess the severity of the lesion
Aiman A. Ali DDS, PhD.

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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. Pemphigus vegetans
 Skin,
vermilion and oral mucosa
 Histopathologically: epithelial hyperplasia
with intraepithelial abscess formation
 Abundant eosinophils
Aiman A. Ali DDS, PhD.

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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. Paraneoplastic Pemphigus
 Simulate
pemphigus vulgaris clinically
 Associated with lymphoma or other
malignancies
 Histopathologically and IF is different
Aiman A. Ali DDS, PhD.

25. Cicatricial
Pemphigoid
Aiman A. Ali DDS, PhD.

26. 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.

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28. 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.

29. 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.

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32. 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.

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36. Differential diagnosis
Pemphigus
vulgaris
Erosive lichen planus
Aiman A. Ali DDS, PhD.

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38. Treatment
 Topical
corticosteroids (betamethasone
dexamethasone…etc)
 In severe cases systemic SAIDs with
immunosuppressive agents
Aiman A. Ali DDS, PhD.

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40. Bullous
pemphigoid
Aiman A. Ali DDS, PhD.

41. 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

42. Clinical features
 Very
common in the 7th and 8th decades
 Lesions
affect the skin

43. Histopathology
 Normal HP
the same of CP
 Ultrastructurally:
the basement membrane
is cleaved at the level of lamina lucida

44. 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

45. Treatment
 Systemic
corticosteroids

47. Dermatitis
herpetiformis

48. Etiology
 Unknown
cause
 Deposits of
 No
IgA in the skin and mucosa
circulating autoantibodies in the
patient’s serum

49. 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

50. Histopathology
 Accumulation
of neutrophils and eosinophils
producing dermal micro-abscess
 Connective
tissue become necrotic and the
overlying epithelium separate
 Formation
of subepithelial vesicle

51. Immunopathology
 Immunofluorescent
staining is positive
at the epidermal-dermal junction
 Almost IgA
alone or in combination
with IgG or IgM

52. Treatment
 It dose
not respond to SAIDs
 Sulfapyridine is the
 Gluten-free
diet
treatment of choice

54. Linear IgA
Disease

55.  IgA deposits at
the dermal-epidermal
junction in linear pattern
 Not
associated with gluten-sensitive
enteropathy
 Common
oral lesions
 Separation
at the basement membrane

56. Epidermolysis
Bullosa

57. Etiology

Hereditary disease

In one type acquired

Formation of blisters at sites of minor trauma

58. 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)

59. Treatment
Symptomatic

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