vesicobullous diseases inludes Viral & imune mediated disease i hope these slides ill help retain this topic

1. VesiculoBullous Diseases

2.  A vesiculobullous disease is a type of mucocutaneous disease that is
characterized by vesicles and bullae (i.e. blisters).
 Both vesicles and bullae are fluid-filled lesions, and they are distinguished
by size (vesicles being less than 5-10 mm and bulla being larger than 5-10
mm)

3. Classification:-
Viral diseases
 Herpes simplex infection
 Varicella-Zoster infection

4. Classification:-
Immunologic Disease
 Pemphigus Vulgaris
 Mucous membrane pemphigoid (MMP)
 Bullous Pemphigoid (BP)
 Dermatitis Herpetiformis
 Linear Immunoglobulin A disease (lAD)

5. Classification:-
Hereditary Disease
 Epidermolysis Bullosa
 Angina bullosa heamorrhagica

6. Herpes Simplex Infection
 Common vesicular eruption of skin and mucosa
 Forms:-
 HSV-1
 1) Primary (Acute primary herpetic gingivostomatitis)
 2) secondary or recurrent (Herpes Labialis / coldsores)
 HSV-2 (Genital Herpes)

7. Herpes Simplex Infection
Pathogenesis:-
Host(Seronegative)
Direct Contact with HSV
Primary Disease
Gingivostomatitis
Secondary disease (lip, gingiva,palate)
Host seropositive
Latent virus in Nerve Ganglia
reactivation

8. Acute Primary Herpetic Gingivostomatitis
 Most common Viral Infection affecting mouth
 Usually seen in pre-school children , teenagers
 Clinical Features:-
 Prodromal features ( Headache, fever, malaise, lymphadenopathy, skin rash)
 Oral and perioral vesicles appear & they rupture to form ulcers
 Oral ulcerations (typically all oral mucosa)
 Gingiva become boggy, ulcerated @ free margins, pain
 Course = 7-10days
 Lesions heal without scar formation

10. Viral Diseases
Case Presentation 1:-
 A child 10yrs old came to our clinic & presented with fever, sore throat, headache,
and vesicular eruptions in the oral cavity. On clinical examination his oral mucosa
was bright red , sore , and he had marked gingivitis and his cervical lymph nodes
were palpable

11. Viral Diseases
Case presentation 2:-
A 2 yr old girl reports to you with a complaint of widespread oral ulcerations &
gingival swelling with a history of fever & sore throat for 2 days

12.  What is your DIAGNOSIS????
 How ill You MANAGE the patient????

13. Diagnosis:-
 History
 Clinical Examination
 Additional Investigations:-
 1)Viral swab-culture EM, PCR
 2)Vesicular fluid-tzank cells
 3)Viral Antibody titers (IgM,IgG)

14. DIAGNOSIS
“Primary Herpetic Gingivostomatitis”
MANAGEMENT
In childhood
a) Antipyretics paracetamol
b) Analgesics paracetamol
c) Local analgesics benzydamine HCL m/w or spray, lidocaine gel 1-2%
d) Fluids
e) Antihistamines
f) Monitor temperature

15. MANAGEMENT
 In adults
a) Antipyretics (paracetamol or NSAIDS)
b) Analgesics
c) Fluids
d) Acyclovir-200mg 5x daily for 5 days

16. Secondary Herpes Infection
 Reactivation of the latent herpes virus type 1
 Precipitating factors :-
 Sunlight exposure
 Exposure to cold
 Trauma
 Stress
 Immunosuppression
 Pregnancy
 Other illness

17. Clinical Features
 Prodromal symptoms (Tingling & Burning)
 Mucocutaneous junction of the Lip Pururitis ,reddening , Vesiculation , Pustule ,
Ulcer
 Intraoral recurrences (Hard palate, gingiva)
 Self limited

18. Diagnosis:-
 History
 Clinical examination
 Occasionally identify by culture PCR
 Serology not helpful
Management:-
 Acyclovir cream 5% 4 hourly
 Penciclovir cream 1% 2 hourly

19. Varicella Zoster Infection
 Childhood - Primary infection> Chicken Pox
 Adulthood – Secondary infection > Shingles
Chicken Pox:-
Common disease
Route of spread (direct contact, droplet infection)
Clinical Features:-
Prodromal features
Oral vesicles or Ulcers typically on palate
Skin Rash primarily involve trunk head and neck
Pruritic>papular>vesicular>pustular>crusts>trunk/face esp. affected/first sites

21. Investigations
 History
 Clinical Examination
 Virus identified by culture EM, PCR
Treatment
 Analgesics
 Antipyretics

22. Herpes Zoster(Shingles)
Middle or Older Age
Predisposing Factors (debilitating diseases immunosuppression , cancer )
Clinical Features
 Vesicular Eruption in area of Distribution of sensory nerve
 Band like distribution of shingles on trunk(zoster, girdle)
 Trigeminal Nerve most commonly ophthalmic division involve-corneal ulceration
 C/f in the mouth:-
 Unilateral Vesicles breakdown to form ulcers

23. Ramsay hunt Syndrome:-
 Involvement of Facial in auditory nerve produce ramsay hunt syndroe
 Lower motor neuron facial palsy
 Otitis Externa
 Vesicles of ant 2/3rd tongue
 Vesicles or ulcers of soft palate

24. Investigations:-
 History
 Clinical Examination
 FBC
 Rarely-identify virus (e.g. culture, EM, PCR)
 Serology not helpful
Treatment:-
 Analgesics
 Antiviral agents
 Aciclovir , valaciclovir, famciclovir ,brivudin
 Aciclovir (800mg 5times/day for 7-10days)
 Tetracycline mouth washes (for oral lesions)

25. Viral Diseases
Case presentation
 A 65yr old male presented to clinic with tooth Ache for 2-
3days after that vesicles appear on palate , accompanied by
corneal ulceration. Similar vesicles are aslo present on skin
and form firm crust.

26. Hereditary Diseases:-
 Epidermolysis Bullosa
A generalized desquamating condition of the skin & mucosa with associated
scarring , contractures , & dental defects that occur in 3 main hereditary forms in
children & one acquired form in adults
Types:-
Type Genetic Pattern Location of separation Defective Structure
Hereditary
Simplex Autosomal dominant Intraepithelial Linking proteins
Junctional Autosomal recessive Lamina Lucida Anchoring proteins
Dystrophic Autosomal
dominant/recessive
Sublamina Densa Type 7 collagen
Acquired
Acquista None Sublamina Densa Type 7 collagen

27. Clinical Features:-
 Simplex Epidermolysis Bullosa
 Skin blisters at birth, mainly induced by friction.
 Oral involvement absent or mild.
 Teeth normal
 Site of Blister:- Basal cells
 Inheritance:- Mainlu autosomal dominant

28. Clinical Features:-
 Junctional epidermolysis bullosa
 Lethal form results in extensive skin & mucosal involvement ,
 dental abnormalities
 & often with death in infancy.
 The nonlethal form produces widespread skin & variable mucosal involvement
 Intraorally large fragile heamorragic blisters of palate
 Crusted granular heamorrahagic lesions present in perioral and perinasal

29. Clinical Features:-
 Dystrophic Epidermolysis Bullosa
 Dominant form is often mild.
 Recessive form is very severe with extensive blisters & scarring of skin,
 Loss of nails
 Severe oral mucosal blistering & scarring results in diminished opening
 Hypoplastic teeth
 Epidermolysis bullosa acquisita
 Trauma or friction induced blisters
 Intraoral blisters rare
 If present, they produce scarring & diminished oral opening

30. Management:-
 No specific TX available!
 Avoidance of trauma
 Prevention of infections
 Systemic use of phenytoin
 Corticosteroids (high doses )
 Systemic use of phenytoin (Dilantin)
 Vitamin E
 Retinoids
 Dapsone
 Maintaninance of nutritional status is required in case of restricted oral opening

32. Idiopathic oral blood blisters(Angina bullosa
hemorrhagica)
 Formation of spontaneous blood filled bullae (blood blisters) of oral mucosa
 Both GENDERS affected
 Clinical Features
 Sharp pricking sensation
 Most commonly on palate
 Bullae may be quite large 2-3cm
 Commonly occur when pt is eating

34. Mechanism:-
 Bleeding from the capillary bed below a basal zone that is for some reason
weakend
 Pt with thrombocytopenia may develop blood blisters
Diagnosis:-
History
Full blood count
Clotting screen
Management:-
Perforation of large intact blister is necessary
Antiseptic mouth washes (Chlorhexidine gluconate)
Analgesic mouth washes (Benzydamine HCL)
No preventive TX

35. What is an Immunologic Disease ?
A disease resulting from a disordered immune reaction in
which antibodies are produced against one's own tissues, as
systemic lupus erythematosus or rheumatoid arthritis.

36. Immunologic Disease
 Pemphigus Vulgaris
 Immunobullous disorder
 Middle-age disease (40-60yr)
 Affects :- Skin & Mucous membrane
 50% cases begin in mouth (First to SHOW last to GO)
 Characterized by :-
1)Intraepithelial Blister formation
2)Painful ulcers preceded by bulla
3)Bullae are fragile & breakdown rapidly to form CRUSTED,
ERODED lesions
4)Ulcers range from small APTHOUS like to large MAP
like lesions

37. Nikolsky’s Sign
 Gentle traction on clinically unaffected mucosa may produce stripping of
epithelium, a +ve nikolsky sign

38. Pathogenesis:-
 Circulating autoantibodies IgG
Reactive against epithelial desmosome-tonofilament complexes
(Desmoglein 3)
result in dissolution or disruption of intercellular junctions & loss of
cell to cell adhesion

39. Types:-
 Pemphigus Vulgaris
 Pemphigus Foliacues
 Pemphigus Erythematous
 Pemphigus Vegetans
 IgA type
 Drug Induced
Vulgaris/vegetans Affect the Whole Epithelium
Erythematous/Foliaceus Upper Prickle cell layer

40. Intraoral Blisters

41. Diagnosis:-
 History
 Clinical examination
Red painful ulcers
Bullae formation
+ve nikolsky’s sign
small vesicles on soft palate, buccal mucosa, floor of the mouth
 Immunofluorescence
(DIF) Fish-net appearance (IgG, C3 )
(IDIF) +ve IgG 90%
 ELISA

42.  These changes are seen in the STRATUM SPINOSUM or THE PRICKLE CELL LAYER
 The basal cells loose their intercellular bridges but they remain attach to the
dermis, giving a TOMBSTONE appearance
 The blister cavity contains acantholytic cells which often show degenerative
changes

43. Tzank cells

44. Intraoral image:-

45. D/D
 MMP
 EM
 Erosive lichen planus
 Paraneoplastic pemphigus
 Apthous Ulcers

46. Management
 Systemic Corticosteroids
A) Azathioprine
B) Methotrexate
C) Cyclophosphamide
D) Cyclosporine
A) Other:-
B) Mycophenolate mofetil
C) Plasmapheresis In sick people,
plasma can contain antibodies that
attack the immune system.
D) Levamisole (Discontinued)
E) Immunoglobulins
F) High dose iv Ig
G) Infliximab

47. TX Monitoring
Weight Blood pressure

48. TX Monitoring
Blood glucose LFT’s
Full blood count

49. TX Monitoring
Renal function test Cataracts

50. Mucous membrane pemphigoid
 Chronic blistering disease
 Affects:- oral & ocular mucous membrane
 Adults and elderly (affect women more than men)
 Characterized by:-
 Sub-epithelial bulla formation
 intraoral intact bullae present on gingiva or soft palate
 ulcerated area of mucosa involve buccal, palatal or lip mucosa
 desquamative gingivitis
 lesions are chronic & persistent heal with a scar (cicatrix) particularly lesions
of eye

51. Usually Buccal mucosa Gingiva & soft palate

52. Types:-
 Cicatrical Pemphigoid
 Benign mucous membrane pemphigoid
 Ocular Pemphigus
 Childhood Pemphigoid
 Mucosal Pemphigoid
 Gingivosis

53. Risks include:- Symblephron, entropion,
trichiasis

54. Pathogenesis:-
 Auto-Antibodies IgG
Target antigen BP 180 & laminin 5
Result in sub-epithelial clefting

55. D/D
 Pemphigus vulgaris
 Erosive lichen planus
 If attatched gingiva involve then ;
atrophic lichen planus , linear igA disease , contact allergy , DLE

56. Diagnosis
 History (Drug history, drug induced)
 Biopsy (Sub epithelial blister formation)
 Direct Immunofluorescence (linear IgG @ BM)
 Indirect Immunofluorescence (Pemphigoid antibodies)

57. Management
 Topical corticosteroids
 Systemic corticosteroids
 Azathriopine
 Dapsone (diaminodiphenylsulfone) 25 mg & 100 mg Tablets for Oral
Use
 High dose IV ig
 Referral for Eyes, skin, larynx, genitalia

58. Bullous Pemphigoid
 Sub epidermal blisters
 Elder 7th & 8th decade
 Involves Skin & mucous membrane
 Characterized by:-
 Skin lesions (Tense vesicles on trunk and limbs) preceded by erythematous
popular eruption
 Oral lesions (bullae & erosions mostly noted on attached gingiva)
 Other sites:- soft palate , buccal mucosa & floor of mouth

60. Diagnosis
 History
 Biopsy demonstrate IgG or c3 in a linear distribution
 Main target antigen BP 230 & BP 180
 BP 230 & BP 180 Important proteins in maintaining the structural
integrity of the basement membrane i.e. in providing COHESION of the dermis
to the epithelium

61. Management
 Systemic corticosteroids
Prednisolone 0.75-1mg/kg daily
As soon as, control is reached tapering to maintenance dose of 8mg daily
 Topical Corticosteroids
Azathioprine
Cyclophosphamide
Mycophenolate also appear promising

62. Dermatitis Herpetiformis
 Cutaneous disease
 Young and middle age adults
 More common in males
 Associated with Gluten sensitive enteropathy –celiac disease
 Characterized by:-
 Cutaneous lesions:- Papular, erythematous, vesicular, intensely pruritic
 (extensor surfaces of elbow, shoulder, sacrum, buttocks)
 Frequent involvement of scalp and face
 Oral cavity (uncommon vesicles and bullae that rupture leaving superficial ulcers)

64. Pathogenesis:-
 Auto-Antibodies IgA
Binds with Epidermal transglutaminase

65. Diagnosis
History
Biopsy (fluid filled sub epithelial bullae
Direct immunofluorescence – Granular deposits of IgA at BM
Serology for Celiac disease- As the disease gets worse, more
antibodies will be present. If a disease is suspected, the test
may need to be repeated 10 days to 2 weeks after the first
test.
Full blood count
LET’S may be abnormal

66. Management
 Dapsone 50 mg orally once a day continued on a life-long basis. Dosage
may be advanced to 300 mg/day. Dosage reduction to a minimum
maintenance level as soon as possible is recommended.
 Sulfoxone rapidly absorbed
 Sulfapyridine
 Gluten free diet

67. Linear IgA disease
 Rare bullous disease of skin
 2 Types:-
 1)Affecting children (chronic bullous disease of childhood)
 2)Adults (Adult linear IgA disease)
 Characterized By:-
 Skin lesions Tense bullae on trunk, limbs or scalp (ROSETTES)
 Oral lesions Ulcerative or erosive similar to those seen in pemphigoid

69.  Drug Induced Linear igA
 Vancomycin
 Diclofenac
 Captopril
 Cotrimoxazole
 Amiodarone
 Cyclosporine
 Glibencamide
 Lithium
 Penicillin
 Cefamandole
 Phenytoin
 Somatostatin
 Sodium hypochlorite (bleach)

70. Management
 Gluten free diet
 Topical corticosteroids
 Dapsone
 Sulfapyridine
 Referral regarding celiac disease