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Vesiculobullous diseases

Vesiculobullous diseases. A vesiculobullous disease is a type of mucocutaneous disease that is characterized by vesicles and bullae (i.e. blisters ).

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Vesiculobullous diseases

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  1. Vesiculobullousdiseases

  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 mm and bulla being larger than 5 mm)

  3. CLASSIFICATION OF VESICULOBULLOUS DISEASES CLASSIFICATION INTRA EPITHELIAL VESICLES: The lesion is formed within the epithelium • Acantholytic vesicles : This is because of the break down of specialized attachments called the desmosomes • Nonacantholytic vesicles: It is usually in the viral infections because of the death or the rupture of the group of cells. SUB EPITHELIAL VESICLES: Lesions formed between the epithelium and the lamina propriaeg: • Erthymamultifome • Phempegoid • Dermatitis herpetiformis • Epidermolysisbullosa

  4. Classification:- • Viral diseases • Herpes simplex infection • Varicella-Zoster infection • Immunologic Disease • PemphigusVulgaris • Mucous membrane pemphigoid (MMP) • BullousPemphigoid (BP) • Dermatitis Herpetiformis • Hereditary Disease • EpidermolysisBullosa • Angina bullosaheamorrhagica

  5. What is an Immunologic Disease ? Adisease resulting from A disorderedimmune reaction in which antibodies are produced against one's own tissues, as systemic lupus erythematosus or rheumatoid arthritis.

  6. PEMPHIGUS • It is an autoimmune disease • There are circulating antibodies of type IgG. • These antibodies are reactive against the desmosomes or the tonofilament complex. • There destruction or disruption of these tonofilament complex ,resulting in the loss of attachment from cell to cell

  7. Epithelial attachment apparatus.

  8. Types:- • Pemphigus Vulgaris • Pemphigus Foliacues • Pemphigus Erythematous • Pemphigus Vegetans Vulgaris/vegetans Affect the Whole Epithelium Erythematous/Foliaceus Upper Prickle cell layer

  9. PEMPHIGUS VULGARIS • The epithelial damage is directly proportion to the number of the circulating antibobies. • The tonofilament or desmosomes are disrupted by a proteolytic enzyme which is released by these antibodies .

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

  11. 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 APTHOUSlike to large MAP like lesions

  12. Pemphigusvulgaris. Multiple erosions of the left buccal mucosa. Pemphigusvulgaris. Large, irregularly shaped ulcerations involving the floor of the mouth and ventral tongue.

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

  14. Pemphigusvulgaris. This flaccid cutaneousbulla is characteristic of skin involvement. Pemphigusvulgaris. Multiple erosions affecting the marginal gingiva.

  15. PEMPHIGUS VULGARIS

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

  17. Intraoral Blisters

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

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

  20. PEMPHIGUS VULGARIShistology

  21. PEMPHIGUS VULGARIShistology

  22. 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

  23. Tzank cells

  24. PEMPHIGUS VULGARIStazank cells

  25. Intraoral image:-

  26. D/D • MMP • EM • Erosive lichen planus • Paraneoplastic pemphigus • Apthous Ulcers

  27. PEMPHIGUS VULGARIS TREATMENT: • High mortality rates previously • Introduction of systemic corticosteroids like prednisolone in stable cases. • Prednisolone plus azathioprinemethotrexate and cyclophospamide in progressed or advance cases.

  28. PEMPHGOID • Mucous membrane pemphigoid (cicatricial) CIKA-TRI-CIAL • Bullouspemphigoid

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

  30. PEMPHGOID • CASCADEOF EVENTS Antibody antigen complex  Complement activation  Neutrophils & Eosinophils recruited  Release of proteases by the recruited cells  Sub epithelial blister formation

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

  32. 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

  33. Usually Buccal mucosa Gingiva & soft palate

  34. PEMPHGOID

  35. PEMPHGOID CLINICAL FEATURES(MMP) • Oral mucosa is the first site- lesions are rarely wide spread • Subepithelialbullae, ruptured in the later stages. • Bleeding in the bullae – bleeding blisters • Slow progress, skin involvement absent or rare • Involvement of eyes, nose larynx, pharynx and osephaghus.

  36. PEMPHGOIDoccular involvement

  37. Mucous membrane pemphigoid. Although cutaneous lesions are not common, tense bullae such as these may develop on the skin of 20% of affected patients.

  38. Mucous membrane pemphigoid. One or more intraoral vesicles, as seen on the soft palate. Usually, ulcerations of the oral mucosa are also present. Mucous membrane pemphigoid. Large, irregular oral ulcerations characterize the lesions after the initial bullae rupture.

  39. Mucous membrane pemphigoid. Often the gingival tissues are the only affected site, resulting in a clinical pattern known as desquamative gingivitis. Such a pattern may also be seen with lichen planus and pemphigusvulgaris.

  40. PEMPHGOID

  41. Mucous membrane pemphigoid. Mediumpower photomicrograph of perilesional tissue shows characteristic subepithelialclefting. Mucous membrane pemphigoid. Direct immunofluorescence studies show a deposition of immunoreactants at the basement membrane zone of the epithelium

  42. D/D • Pemphigus vulgaris • Erosive lichen planus • If attatched gingiva involve then ; atrophic lichen planus, contact allergy

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

  44. Management • Topical corticosteroids • Systemic corticosteroids • Azathriopine • Dapsone (diaminodiphenylsulfone) 25 mg & 100 mg Tablets for Oral Use • Referral for Eyes, skin, larynx, genitalia

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

  46. BULLOUS PEMPHGOID

  47. Bullouspemphigoid. These oral lesions appear as large, shallow ulcerations involving the soft palate.

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