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.
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.
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.
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.
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.
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.
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
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
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
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
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)