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Vesiculo bullous II

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Oral Pathology I …

Oral Pathology I
Third Year

Published in: Health & Medicine, Technology

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  • 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.
  • 7. Aiman A. Ali DDS, PhD.
  • 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.
  • 10. Aiman A. Ali DDS, PhD.
  • 11. Tzanck cells Aiman A. Ali DDS, PhD.
  • 12. Aiman A. Ali DDS, PhD.
  • 13. Aiman A. Ali DDS, PhD.
  • 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.
  • 16. Aiman A. Ali DDS, PhD.
  • 17. Aiman A. Ali DDS, PhD.
  • 18. Aiman A. Ali DDS, PhD.
  • 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.
  • 21. 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.
  • 23. Paraneoplastic Pemphigus  Simulate pemphigus vulgaris clinically  Associated with lymphoma or other malignancies  Histopathologically and IF is different Aiman A. Ali DDS, PhD.
  • 24. Cicatricial Pemphigoid Aiman A. Ali DDS, PhD.
  • 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. { { { { { {
  • 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. 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. Aiman A. Ali DDS, PhD.
  • 30. Aiman A. Ali DDS, PhD.
  • 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. Aiman A. Ali DDS, PhD.
  • 33. Aiman A. Ali DDS, PhD.
  • 34. Aiman A. Ali DDS, PhD.
  • 35. Differential diagnosis Pemphigus vulgaris Erosive lichen planus Aiman A. Ali DDS, PhD.
  • 36. Aiman A. Ali DDS, PhD.
  • 37. Treatment  Topical corticosteroids (betamethasone dexamethasone…etc)  In severe cases systemic SAIDs with immunosuppressive agents Aiman A. Ali DDS, PhD.
  • 38. Aiman A. Ali DDS, PhD.
  • 39. Bullous pemphigoid Aiman A. Ali DDS, PhD.
  • 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. Clinical features  Very common in the 7th and 8th decades  Lesions affect the skin
  • 42. Histopathology  Normal HP the same of CP  Ultrastructurally: the basement membrane is cleaved at the level of lamina lucida
  • 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. Treatment  Systemic corticosteroids
  • 45. Dermatitis herpetiformis
  • 46. Etiology  Unknown cause  Deposits of  No IgA in the skin and mucosa circulating autoantibodies in the patient’s serum
  • 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. Histopathology  Accumulation of neutrophils and eosinophils producing dermal micro-abscess  Connective tissue become necrotic and the overlying epithelium separate  Formation of subepithelial vesicle
  • 49. Immunopathology  Immunofluorescent staining is positive at the epidermal-dermal junction  Almost IgA alone or in combination with IgG or IgM
  • 50. Treatment  It dose not respond to SAIDs  Sulfapyridine is the  Gluten-free diet treatment of choice
  • 51. Linear IgA Disease
  • 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. Epidermolysis Bullosa
  • 54. Etiology  Hereditary disease  In one type acquired  Formation of blisters at sites of minor trauma
  • 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. Treatment Symptomatic
  • 57. { { { { { {