Vesiculobullous diseases

1. Vesiculobullous Vesiculobullous
diseasesdiseases
PEMPHIGUS VULGARISPEMPHIGUS VULGARIS
PEMPHGOIDPEMPHGOID
ERYTEMA MULTIFORMEERYTEMA MULTIFORME
EPIDERMOLYSIS BULLOSAEPIDERMOLYSIS BULLOSA
LUPUS ERYTHEMATOSISLUPUS ERYTHEMATOSIS
Dr.khurram zafar Dr.khurram zafar

2. CLASSIFICATION OF VESICULOBULLOUS CLASSIFICATION OF VESICULOBULLOUS
DISEASESDISEASES
VESICLE&BULLAVESICLE&BULLA
A clear fluid lesion just below the A clear fluid lesion just below the
epithelium which ruptures to form an ulcer, epithelium which ruptures to form an ulcer,
if this is smaller than 5mm then it is a if this is smaller than 5mm then it is a
vesicle ,if larger than 5mm than it is a bulla vesicle ,if larger than 5mm than it is a bulla

3. CLASSIFICATION OF VESICULOBULLOUS CLASSIFICATION OF VESICULOBULLOUS
DISEASESDISEASES
CLASSIFICATIONCLASSIFICATION
INTRA EPITHELIAL VESICLESINTRA EPITHELIAL VESICLES: The lesion is formed : The lesion is formed
within the epithelium within the epithelium
Acantholytic vesicles : Acantholytic vesicles : This is because of the break This is because of the break
down of specialized attachments called the down of specialized attachments called the
desmosomes desmosomes
Nonacantholytic vesiclesNonacantholytic vesicles: It is usually in the viral : It is usually in the viral
infections because of the death or the rupture of the infections because of the death or the rupture of the
group of cells.group of cells.
SUB EPITHELIAL VESICLESSUB EPITHELIAL VESICLES: Lesions formed between the : Lesions formed between the
epithelium and the lamina propria eg:epithelium and the lamina propria eg:
Erthyma multifomeErthyma multifome
PhempegoidPhempegoid
Dermatitis herpetiformisDermatitis herpetiformis
Epidermolysis bullosaEpidermolysis bullosa

4. PEMPHIGUS VULGARISPEMPHIGUS VULGARIS
Autoimmune disease.Autoimmune disease.
Common in Ashkenazi and Mediterranean jews .Common in Ashkenazi and Mediterranean jews .
Middle aged females.Middle aged females.
Other variants are:Other variants are:
Pemphius VegitansPemphius Vegitans
Pemphigus Foliaceus & ErthematosusPemphigus Foliaceus & Erthematosus
Paraneoplastic pemphigus.Paraneoplastic pemphigus.

5. PEMPHIGUS VULGARISPEMPHIGUS VULGARIS
CLINICAL FEATURES:CLINICAL FEATURES:
Painful ulcers or bulla are formed which are fluid Painful ulcers or bulla are formed which are fluid
filled. filled.
They can be formed any where in the oral cavity .They can be formed any where in the oral cavity .
The bulla is rapidly ruptured leaving a collapsed roof The bulla is rapidly ruptured leaving a collapsed roof
of grayish membrane with a red ulcerated base.The of grayish membrane with a red ulcerated base.The
ulcer may look like an apthous ulcer or may be large ulcer may look like an apthous ulcer or may be large
map shaped.map shaped.
Nikolsky sign is positive.Nikolsky sign is positive.
C/F con…dC/F con…d

6. PEMPHIGUS VULGARISPEMPHIGUS VULGARIS
Some time the ulcers are joined together to make a Some time the ulcers are joined together to make a
confluence this condition is very painful.confluence this condition is very painful.
It has a variable course might involve skin, It has a variable course might involve skin,
oesophagus, cervix. oesophagus, cervix.
Protein/fluid,electrolyte and weight loss /secondary Protein/fluid,electrolyte and weight loss /secondary
infections.infections.
Fatal if untreated. Fatal if untreated.

7. PEMPHIGUS VULGARISPEMPHIGUS VULGARIS

8. PEMPHIGUS VULGARISPEMPHIGUS VULGARIS
PATHOGENESIS:PATHOGENESIS:
It is an autoimmune diseaseIt is an autoimmune disease
There are circulating antibodies of type IgG.There are circulating antibodies of type IgG.
These antibodies are reactive against the These antibodies are reactive against the
desmosomes or the tonofilament complex.desmosomes or the tonofilament complex.
There destruction or disruption of these There destruction or disruption of these
tonofilament complex ,resulting in the loss of tonofilament complex ,resulting in the loss of
attachment from cell to cellattachment from cell to cell
path.cont…dpath.cont…d

9. PEMPHIGUS VULGARISPEMPHIGUS VULGARIS
The epithelial damage is directly proportion to The epithelial damage is directly proportion to
the number of the circulating antibobies.the number of the circulating antibobies.
The tonofilament or desmosomes are disrupted The tonofilament or desmosomes are disrupted
by a proteolytic enzyme which is released by by a proteolytic enzyme which is released by
these antibodies .these antibodies .
The cell to cell break down also takes place The cell to cell break down also takes place
through a complement system but this process through a complement system but this process
is not clearly understood . is not clearly understood .

10. PEMPHIGUS VULGARISPEMPHIGUS VULGARIS

11. PEMPHIGUS VULGARISPEMPHIGUS VULGARIS
HISTOPATHOLOGY:HISTOPATHOLOGY:
Intra epithelial vesicles or bulla and cleft like spaces Intra epithelial vesicles or bulla and cleft like spaces
are produced by acantolysis are produced by acantolysis
These changes are in the stratum spinosum or the These changes are in the stratum spinosum or the
prickle cell layerprickle cell layer
The basal cell remain attach to the lamina propria The basal cell remain attach to the lamina propria
and project into the bulla like tombstones.and project into the bulla like tombstones.
Inflammatory cells are very scanty however Inflammatory cells are very scanty however
eosinophils may be seen.eosinophils may be seen.
Acantholytic statum spinosum cells occur singly or Acantholytic statum spinosum cells occur singly or
are in the forms of clumps lying freely within the are in the forms of clumps lying freely within the
blister fluid. These cell loose there polyhedral blister fluid. These cell loose there polyhedral
morphology rather they are small rounded and morphology rather they are small rounded and
contain hyper chromatic nuclei called the TAZANK contain hyper chromatic nuclei called the TAZANK
CELLS.CELLS.

12. PEMPHIGUS VULGARISPEMPHIGUS VULGARIS
histologyhistology

13. PEMPHIGUS VULGARISPEMPHIGUS VULGARIS
histologyhistology

14. PEMPHIGUS VULGARISPEMPHIGUS VULGARIS
tazank cellstazank cells

15. PEMPHIGUS VULGARISPEMPHIGUS VULGARIS
immunoflorecenceimmunoflorecence

16. PEMPHIGUS VULGARISPEMPHIGUS VULGARIS
DIFFRENTIAL DIAGNOSIS:DIFFRENTIAL DIAGNOSIS:
Pempegiod Pempegiod
Erthema multiformeErthema multiforme
Bullous lichen plannus Bullous lichen plannus

17. PEMPHIGUS VULGARISPEMPHIGUS VULGARIS
TREATMENT:TREATMENT:
High mortality rates previously High mortality rates previously
Introduction of systemic corticosteroids Introduction of systemic corticosteroids
like prednisolone in stable cases.like prednisolone in stable cases.
Prednisolone plus azathioprine Prednisolone plus azathioprine
methotrexate and cyclophospamide in methotrexate and cyclophospamide in
progressed or advance cases. progressed or advance cases.

18. PEMPHGOIDPEMPHGOID

19. PEMPHGOIDPEMPHGOID
Mucous membrane pemphigoid Mucous membrane pemphigoid
(cicatricial) CIKA-TRI-CIAL(cicatricial) CIKA-TRI-CIAL
Bullous pemphigoid Bullous pemphigoid

20. PEMPHGOIDPEMPHGOID
PATHOLOGYPATHOLOGY
Autoimmune diseaseAutoimmune disease
Not life threateningNot life threatening
Elderly females above 60 yrs of ageElderly females above 60 yrs of age
Loss of attachment and separation of full thickness Loss of attachment and separation of full thickness
epithelium from the lamina propria.epithelium from the lamina propria.
Alteration of rete pegsAlteration of rete pegs
Epithelium forms the roof of the blistersEpithelium forms the roof of the blisters
Auto antibodies are formed against the Auto antibodies are formed against the
hemidesmosomes (BPAG-1,230kd;BPAG-2; 180kd.hemidesmosomes (BPAG-1,230kd;BPAG-2; 180kd.
Inflammatory Inflammatory
cells(lymphocytes,neutrophils,eosinophils)are seen cells(lymphocytes,neutrophils,eosinophils)are seen
in the later stagesin the later stages

21. PEMPHGOIDPEMPHGOID

22. PEMPHGOIDPEMPHGOID
CLINICAL FEATURES(MMP) CLINICAL FEATURES(MMP)
Oral mucosa is the first site- lesions are rarely Oral mucosa is the first site- lesions are rarely
wide spreadwide spread
Subepithelial bullae, ruptured in the later stages.Subepithelial bullae, ruptured in the later stages.
Bleeding in the bullae – bleeding blistersBleeding in the bullae – bleeding blisters
Slow progress, skin involvement absent or rareSlow progress, skin involvement absent or rare
Involvement of eyes, nose larynx, pharynx and Involvement of eyes, nose larynx, pharynx and
osephaghus osephaghus
Nikolsky sign is positive Nikolsky sign is positive

23. PEMPHGOIDPEMPHGOID
occular involvementoccular involvement

24. PEMPHGOIDPEMPHGOID
CASCADEOF EVENTSCASCADEOF EVENTS
Antibody antigen complexAntibody antigen complex
¯¯
Complement activationComplement activation
¯¯
Neutrophils & Eosinophils recruited Neutrophils & Eosinophils recruited
¯¯
Release of proteases by the recruited cellsRelease of proteases by the recruited cells
¯¯
Sub epithelial blister formationSub epithelial blister formation

25. PEMPHGOIDPEMPHGOID

26. PEMPHGOIDPEMPHGOID
MANAGEMENTMANAGEMENT
Confirm diagnosisConfirm diagnosis
Topical corticosteroidsTopical corticosteroids
Ocular involvement –systemic steroids.Ocular involvement –systemic steroids.

27. ERYTEMA ERYTEMA
MULTIFORMEMULTIFORME

28. ERYTEMA MULTIFORMEERYTEMA MULTIFORME
Mucocutaneous diseaseMucocutaneous disease
Males adolosents , young adults are Males adolosents , young adults are
affected moreaffected more

29. ERYTEMA MULTIFORMEERYTEMA MULTIFORME
AETIOLOGY /PATHOLOGYAETIOLOGY /PATHOLOGY
Unclear aetiology and pathogenesisUnclear aetiology and pathogenesis
Infections like HSV can trigger this Infections like HSV can trigger this
diseasedisease
Drugs like Sulphonamides ,barbituratesDrugs like Sulphonamides ,barbiturates
Suggested cause is also given as to a Suggested cause is also given as to a
type III hypersensitivity reactiontype III hypersensitivity reaction

30. ERYTEMA MULTIFORMEERYTEMA MULTIFORME
CLINICAL FEATURESCLINICAL FEATURES
Prodomal signs:Prodomal signs:
Upper respiratory infectionUpper respiratory infection
Headache and malaise Headache and malaise
Nausea and arthralgiaNausea and arthralgia
C/Fcont…dC/Fcont…d

31. ERYTEMA MULTIFORMEERYTEMA MULTIFORME
Signs during the disease:Signs during the disease:
Red macules – 1cm or more in diameter with Red macules – 1cm or more in diameter with
cyanotic centercyanotic center
Lips grossly swollen ,split crusted bleedingLips grossly swollen ,split crusted bleeding
Widespread fibrin covered erosions and Widespread fibrin covered erosions and
erythema in the mouth.erythema in the mouth.
Mild feverMild fever
Conjunctivitis may be associated Conjunctivitis may be associated
Attacks recur at the intervals of several monthsAttacks recur at the intervals of several months
Usually self limiting.Usually self limiting.

32. ERYTEMA MULTIFORMEERYTEMA MULTIFORME

33. ERYTEMA MULTIFORMEERYTEMA MULTIFORME
HISTOPATHOLOGYHISTOPATHOLOGY
Necrosis of the kertinocytesNecrosis of the kertinocytes
Inter & intra cellular odema.Inter & intra cellular odema.
Subepithelial blisters are commonSubepithelial blisters are common
Infiltration of inflammatory cells.Infiltration of inflammatory cells.

34. ERYTEMA MULTIFORMEERYTEMA MULTIFORME

35. ERYTEMA MULTIFORMEERYTEMA MULTIFORME
MANAGEMENTMANAGEMENT
No specific treatment required , if HSV inf.. No specific treatment required , if HSV inf..
acycoviracycovir
Systemic steroids may give relief to the Systemic steroids may give relief to the
fever.fever.
In severe cases antibiotics are used to In severe cases antibiotics are used to
prevent ant secondary infections.prevent ant secondary infections.
Symptomatic –analgesics, antipyretics, Symptomatic –analgesics, antipyretics,
antihistamines. antihistamines.

36. EPIDERMOLYSIS EPIDERMOLYSIS
BULLOSABULLOSA

37. EPIDERMOLYSIS BULLOSAEPIDERMOLYSIS BULLOSA
Definition:Definition:
A large group of clinically similar A large group of clinically similar
desquamating disease processes of the desquamating disease processes of the
skin and mucosa that have in common the skin and mucosa that have in common the
separation of the epithelium from the separation of the epithelium from the
underlying connective tissue and the underlying connective tissue and the
formation of large blisters that frequently formation of large blisters that frequently
result in extensive and often immobilizing result in extensive and often immobilizing
scar formation.scar formation.

38. EPIDERMOLYSIS BULLOSAEPIDERMOLYSIS BULLOSA
MAJOR CATEGORIES OF EPIDERMOLYSIS BULLOSAMAJOR CATEGORIES OF EPIDERMOLYSIS BULLOSA
TypeType Genetic PatternGenetic PatternSeparation LevelSeparation LevelDefec. Structure Defec. Structure
HereditaryHereditary
SimplexSimplex Autosomal dominant Intraepithelial Autosomal dominant Intraepithelial linking proteinslinking proteins
JunctionalJunctional autosomal recessive lamina lucidaautosomal recessive lamina lucida anchoring filamentsanchoring filaments
DystrophicDystrophic autosomal dominant sublamina densa autosomal dominant sublamina densa type VII collagentype VII collagen

AcquiredAcquired
AcquisitaAcquisita None/autoimmune sublamina densaNone/autoimmune sublamina densatype VII collagentype VII collagen

39. EPIDERMOLYSIS BULLOSAEPIDERMOLYSIS BULLOSA
HEREDITARY TYPES:HEREDITARY TYPES:
Congenital absence of componentsCongenital absence of components
ACQUIRED TYPES:ACQUIRED TYPES:
Autoantibodies (IgG; sometimes IgA) to Autoantibodies (IgG; sometimes IgA) to
type VII collagen.type VII collagen.

40. EPIDERMOLYSIS BULLOSAEPIDERMOLYSIS BULLOSA

41. EPIDERMOLYSIS BULLOSAEPIDERMOLYSIS BULLOSA
CLINICAL FEATURESCLINICAL FEATURES
1.1.Epidermolysis Bullosa SimplexEpidermolysis Bullosa Simplex
Mild form; autosomal dominantMild form; autosomal dominant
Sites of trauma/frictionSites of trauma/friction
Involve hands, feet and neck; occ. knees Involve hands, feet and neck; occ. knees
and elbowsand elbows
Teeth not affected; intraoral blisters seenTeeth not affected; intraoral blisters seen
Appears during infancyAppears during infancy

42. EPIDERMOLYSIS BULLOSAEPIDERMOLYSIS BULLOSA

43. EPIDERMOLYSIS BULLOSAEPIDERMOLYSIS BULLOSA
2.2.Junctional Epidermolysis BullosaJunctional Epidermolysis Bullosa
Severe form; autosomal recessiveSevere form; autosomal recessive
Haemorrhagic blisters; loss of nails, large Haemorrhagic blisters; loss of nails, large
blisters of face, trunk and extremitiesblisters of face, trunk and extremities
Generalized scarring and atrophyGeneralized scarring and atrophy
Intraorally-haemorrhagic blisters of palate, Intraorally-haemorrhagic blisters of palate,
perioral and perinasal areasperioral and perinasal areas
Erupted teeth exhibit hypoplastic and severely Erupted teeth exhibit hypoplastic and severely
pitted enamel prone to cariespitted enamel prone to caries

44. EPIDERMOLYSIS BULLOSAEPIDERMOLYSIS BULLOSA
3.3.Dystrophic Epidermolysis BullosaDystrophic Epidermolysis Bullosa
Both autosomal dominant and recessive; recessive is Both autosomal dominant and recessive; recessive is
severesevere
Lesions are birth; arise at pressure sitesLesions are birth; arise at pressure sites
Blisters rupture leaving painful ulcers which heal with Blisters rupture leaving painful ulcers which heal with
large scars that undergo contractures, leading to loss of large scars that undergo contractures, leading to loss of
motility and claw-like hands (Mitten Deformity)motility and claw-like hands (Mitten Deformity)
Teeth exhibit delayed eruption and enamel hypoplasia Teeth exhibit delayed eruption and enamel hypoplasia
with rapid caries developmentwith rapid caries development
Scarring around mouth leads to diminished opening, Scarring around mouth leads to diminished opening,
ankyloglossiaankyloglossia

45. EPIDERMOLYSIS BULLOSAEPIDERMOLYSIS BULLOSA
Epidermolysis Bullosa AcquisitaEpidermolysis Bullosa Acquisita
Non-hereditary form; appears in adulthoodNon-hereditary form; appears in adulthood
Clinically resembles autosomal dominant Clinically resembles autosomal dominant
type of JEB-type VII collagentype of JEB-type VII collagen
Trauma/friction induced blisters of knees, Trauma/friction induced blisters of knees,
elbows, hands and feet- heal with scarselbows, hands and feet- heal with scars
Intraoral blisters rare- when present same Intraoral blisters rare- when present same
picture same picture as JEBpicture same picture as JEB

46. EPIDERMOLYSIS BULLOSAEPIDERMOLYSIS BULLOSA
HISTOPATHOLOGYHISTOPATHOLOGY
Simplex type exhibits zone of cleavage Simplex type exhibits zone of cleavage
(intra-epithelial) above basal cell layer.(intra-epithelial) above basal cell layer.
Remaining types have sub-epithelial Remaining types have sub-epithelial
separationseparation

47. EPIDERMOLYSIS BULLOSAEPIDERMOLYSIS BULLOSA

48. EPIDERMOLYSIS BULLOSAEPIDERMOLYSIS BULLOSA
MANAGEMENTMANAGEMENT
No specific treatment available for hereditary No specific treatment available for hereditary
typestypes
Acquired form maybe treated with Acquired form maybe treated with
corticosteroids and immuno-suppressantscorticosteroids and immuno-suppressants
Maintenance of pt’s nutritional and oral hygiene Maintenance of pt’s nutritional and oral hygiene
statusstatus
Wound healing techniquesWound healing techniques
Prevention of infectionsPrevention of infections
Systemic use of Phenytoin (also acts as a Systemic use of Phenytoin (also acts as a
collagenase inhibitor)collagenase inhibitor)