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  2. 2. GENODERMATOSES• White sponge nevus• Darrier’s disease• Peutz-Jeghers syndrome• Dyskeratosis congenita• Hereditary benign interepithelial dyskeratosis• Panchyonychia congenita• Hyalinosis cutis et mucosa oris• Pseudoxanthoma elasticum
  3. 3. INFECTIVE• Primary herpetic ginigivostomatitis• Secondary herpetic ginigivostomatitis• Varicella ( chickenpox)• Shingles ( Herpes zoster)• Measles• Hand- foot-mouth disease• Small pox and cat scratch disease• Infectious mononucleosis• Herpangia• Acute lymphonodular phayngitis• AIDS
  5. 5. A. VESICULAR• Erythema multiforme• Pemphigus• Benign mucous membrane pemphigoid• Bullous pemphigoid• Epidermolysis bullosa• Bullous lichen planus
  6. 6. B. NON VESICULAR•Lichen planus•Benign migratory glossitis
  7. 7. C. COLLAGEN DISORDER• Lupus erythematous• Scleroderma• Polyarteritis nodosa• Vasculitis• Ischemic lingual necrosis• Wegner granulomatosis• Midline lethal granuloma
  8. 8. D.DEGENERATIVE AND RELATIVE DISORDER•Amyloidosis•Oral submucous fibrosis•Senile solar elastosis
  9. 9. E . PIGMENTATION•Racial pigmentation•Endocrinopathy•Addison disease•Albert syndrome•Bronze diabetes•Anemia
  10. 10. ERYTHEMA MULTIFORME(EM)• Erythema multiforme is an acute limiting dermatitis characterized by distinctive eruption manifested as the iris or target lesion.• TYPES :a) EM Minor – Localized eruption with no or mild mucosal involvementb) EM Major – Mucosal erosions of raised atypical target lesionsc) STEVEN JOHNSON SYNDROME(SJS) – Mucosal erosions ; widespread distribution of flat atypical targets or purpuric macules
  11. 11. (i)ETIOLOGY• Infectious agents :a.HSV { accounts for 55% of EM major}b. Mycoplasma infectionc. Subclinical infection of HSV {EM minor}• Drugs:Sulfa drugs are most common trigger factors
  12. 12. (ii)CLINICAL FEATURES• Young adults – fourth or fifth decades of life• Common in males• Disease is characterized by occurrence of asymptomatic, vividly erythematous discrete macules, papules or occasionally vesicles and bullae distributed in a rather symmetrical pattern most commonly over the hands and arms, feet and legs , face and neck.
  13. 13. TARGET LESIONS• A concentric ring like appearance of the lesions , resulting from the varying shades of Erythema , occurs in the some cases and has give rise to TARGET or IRIS or BULL’S EYE describing them.• Appears rapidly within a day or two and persist from several days to a few weeks , gradually fading and eventually clearing
  14. 14. ORAL MANIFESTATIONS OF EM• Pain and discomfort• Hyperemic macules , papules or vesicles may become eroded or ulcerated and bleed freely• Common: Tongue , palate , buccal mucosa and gingiva
  15. 15. STEVEN-JOHNSON SYNDROME• A severe Bullous form of Erythema multiforme• It commences with the abrupt occurrence of fever, malaise, photophobia and eruptions of the oral mucosa, genital and skin• Patients usually recover unless they succumb to a secondary infection
  16. 16. Oral manifestations SJS• Severe and painful making mastication impossible• Mucosal vesicles or bullae occur which ruptures leaving it covered with a thick white or yellow exudate• Lips exhibit ulceration with bloody crusting and are painful• Erosions of pharynx also common• Mistaken for ANUG
  17. 17. • EYE LESIONS:a. Photophobiab. Conjunctivitisc. Panophthalmitisd. Keratoconjunctivitis sicca• GENITAL LESIONS:a. Non specific urethritisb. Balnitisc. Vaginal ulcers• Others:Tracheo bronchial ulceration and pneumonia
  18. 18. HISTOPATHOLOGY• In general the lesion show intercellular & intracellular edema of the epithelium with focal micro vesicle formation.• Sometimes the edema results in a pooling of an eosinophilic coagulum within the epithelium.• A generalized diffuse infiltration of both acute & chronic inflammatory cells are seen in the underlying C.T. with vasodilatation of blood vessels• C.T. edema & sub-epithelial cleft.
  19. 19. TREATMENT• If drug reaction is suspected ,it should be withdrawn• Infections should be treated appropriately after culture / serologic tests have been performed• Symptomatic treatment :1. Oral antihistamines2. Local skin care3. Topical steroids4. Soothing mouth washes5. Liquid antiseptics { 0.05% chlorhexidine }• Systemic corticosteroids are controversial
  20. 20. • Chronic skin disease characterized by the appearance of vesicles and bullae , small or large fluid filled blisters that develop in cycle• Characterized by intraepithelial bulla formation• TYPES:1. Pemphigus vulgaris2. Pemphigus foliaceous3. Paraneoplastic Pemphigus• Pemphigus vulgaris is the most common type of oral lesion
  21. 21. (i)MECHANISM• Epithelial cell separation : Binding of IgG antibody to Pemphigus antigen leads to the epithelial cell separation by triggering complement activity . Separation of cell takes place in lower layer of stratum spinosum• Associated factors : Autoimmune disorders like thymoma, myasthenia gravis and multiple autoimmune disorders, it may also be triggered by drug therapy like penicillamine, penicillin etc
  22. 22. (ii)CLINICAL FEATURES• 5th to 6th decades of life• Size : thin walled bullae or vesicles varying in diameter from few mm to several cm on normal skin• Signs : lesion contain thin , watery fluid shortly after development , but may become purulent later . They rapidly break and continue to extend peripherally , leaving behind large denuded skin• Nikolsky sign : Application of pressure in normal skin >>> New lesion appears ; reason: upper layer of skin pulling away from basal layer and prevesicular edema which disrupts the dermal-epidermal junction.
  23. 23. (iii)ORAL MANIFESTATIONS• Sites: Buccal mucosa { reason : it has less intercellular substance and fewer intercellular junctions leading to easier acantholysis} ; palate and gingiva• Onset: classic bullae on non inflamed base > ruptures > shallow ulcers• Symptoms : bleed easily ; severe pain ; unable to eat• Signs: thin layer of epithelium peels easily in a irregular pattern leaving behind denuded base
  24. 24. (iv)HISTOLOGY• Intraepithelial vesicle or bullae just above basal layer producing distinctive suprabasilar split• Prevesicular edema appears which weakens the intercellular bridges and junctions• Loss of cohesiveness {ACANTHOLYSIS} because of which clumps of epithelium are often found lying free in the vesicular space { TZANCK CELLS }• Fluid in the vesicles : variable numbers of PMN leukocytes and lymphocytes• SCARCITY of inflammatory cell infiltrate in CT and vesicular fluid differentiate Pemphigus from other Bullous
  25. 25. TZANCK TEST• Tzanck test, also Tzanck smear, is scraping of an ulcer base to look for Tzanck cells.• Tzanck test is very useful for the diagnosis of PV, particularly in the early stages of oral Pemphigus where a biopsy is uncomfortable to the patient• It reveals multiple acantholytic cells (Tzanck cells). A typical Tzanck cell is a large round keratinocyte with a hypertrophic nucleus, hazy or absent nucleoli, and abundant basophilic cytoplasm.• The basophilic staining is deeper peripherally on the cell membrane ("mourning edged" cells) due to the cytoplasm′s tendency to get condensed at the periphery, leading to a perinuclear halo.
  26. 26. Intercellular staining of epidermal skin cells with fluorescentmarkers in a patient with pemphigus vulgaris using directimmunofluorescence.
  27. 27. Pemphigus foliaceous• Mild form, common in older adults• Bullous ruptures and DRY to leave masses of flakes or scales suggestive of an exfoliative dermatitis or eczemaPemphigus erythematosus• Senear-usher syndrome• Bullae or vesicles concomitant with crusted patches , ultimate terminate to vulgaris of foliaceous• Associated fever or malaiseParaneoplastic Pemphigus• Neoplasm like lymphoma or chronic leukemia• Palmar or plantar bullae appear , which does not occur in other types
  28. 28. (v)MANAGEMENT• Corticosteroids : Topical and systemic prednisolone• Combination therapy : High dose of corticosteroids + immunosuppressive drugs such as cyclosporine or azathropine• Plasmapheresis
  29. 29. • Cicatricial pemphigoid (also known as "Benign mucosal pemphigoid," "Benign mucous membrane pemphigoid" ,"Ocular pemphigus "and "Scarring pemphigoid") is a rare chronic autoimmune sub epithelial blistering disease characterized by erosive skin lesions of the mucous membranes and skin that results in scarring of at least some sites of involvement.• Etiology : Auto antibodies of IgG subclass , particularly IgG4 are associated• Common : Females ; 4th to 5th decades of life ;• Sites: oral and eye lesion ; also affects skin and genitals• Feature : Heals by scar
  30. 30. •Ocular involvement are the most serious complication•Adhesions develop between palpebral and bulbar conjunctivae•Opacity of cornea leads to blindness
  31. 31. HISTOPATHOLOGY• Sub epidermal vesicle or bullae• No acantholysis• Non specific Chronic inflammatory infiltrate in connective tissue chiefly lymphocytes and plasma cells
  32. 32. •An chronic, autoimmune ,subepidermal blistering skindisease that involve mucous membranes.•The antigens are bullous pemphigoid-antigen 1 (BPAG1;230kD) which belongs to a family of genes that includesdesmoplakin and bullous pemphigoid antigen 2 (BPAG2;180kD; Type VII collagen).•The primary lesion of B.P. is a tense blister which ariseson either normal appearing or erythematous skin•At times the lesions may arise on urticarial plaques•Most commonly the lesions are found in the flexuralareas•Oral lesions are far less frequent
  33. 33. ORAL MANIFESTATIONS• Oral blisters if present, are mild and transient in contrast to pemphigus• Rarely involves oral mucosa• Gingival lesions resembles Cicatricial pemphigoid• Appears extremely erythematous and may desquamate• Also occurs on buccal mucosa, palate, floor and tongue• Positive Nikolsky sign
  34. 34. HISTOPATHOLOGY:• The dermal papillae are well preserved but edematous• Unlike cicatricial, basement membrane remains attached to connective tissue• No acantholysis• Vesicles contain a fibrinous exudate admixed with occasional inflamatory cells• Direct immunofluorescent examination remains the standard way to confirm the diagnosis
  35. 35. •Epidermolysis bullosa ( EB) is a group of inherited bullousdisorders characterized by blister formation in response tomechanical trauma•Etiology:Mutation of genes• It classified into three types:1.Epidermolysis bullosa simplex-keratinin genes2.Junctional Epidermolysis bullosa-laminin genes3.Dystrophic Epidermolysis bullosa-type VII collagen
  36. 36. (ii) Signs and symptoms•Blistering of the skin•Tooth decay• Deformed or lack of finger/toe nails Internalblistering in the throat, stomach, and intestines•Scalp blistering and loss of hair•Excessive sweating•Hardening of the skin on the feet or hands Thinskin appearance and white bumps Difficultyswallowing•The symptoms vary from the different types.
  37. 37. (iii) ORAL MANIFESTATIONSSOFT TISSUE MANIFESTATIONS:•Increased fragility•Perioral lesions•Microstomia•Ulcerations•Ankyloglossia•Obliteration of oral vestibuleDENTAL MANIFESTATIONS:• Junctional EB have severe enamel hypoplasia•Risk for dental caries•Severe dental crowding and deep bite
  38. 38. •Histologic sections of skin show separation ofthe epidermis from the dermis in asubepidermal plane.•There is absence of a significant inflammatoryresponse.• The adjacent epidermis is normal.
  39. 39. • Most common mucocutaneous lesion• Chronic inflammatory disease of the oral mucosa and skin• Etiology: Although the cause is not well known, T cell- mediated autoimmune phenomena are involved in the pathogenesis of lichen planus.• Possible etiology for unmasking the antigen are contact allergens in restorations or tooth paste. Lichenoid drug reaction may be due to drugs• Characteristic feature: Bilateral white striations, papules, plaques on the buccal mucosa, tongue and gingiva
  40. 40. (i) CLINICAL FEATURES• Lichen planus affects primarily middle- aged adults, and the prevalence is greater among women.• The classic skin lesions of the cutaneous form of lichen planus can be described as six "Ps": pruritic, polygonal, planar, purple ,papules and plaques
  41. 41. (ii) Signs and symptoms• The lesions may appear as:o Lacy, white, raised patches of tissueso Red, swollen, tender patches of tissueso Open sores•LocationoInside of the cheeks, the most common locationoGumsoTongueoInner tissues of the lipsoThroatoEsophagus• Pain or discomfortThe red, inflamed lesions and open sores of oral lichen planuscan cause a burning sensation or pain. The white, lacy patchesalone usually dont cause discomfort, except when they appearon the tongue.
  42. 42. •Other signs or symptomsoA metallic taste or a blunted taste sensation if the tongue isaffectedoDry mouthoDifficulty swallowing if the throat or esophagus is affectedoSensitivity to hot or spicy foodsoBleeding and irritation with tooth brushingSkin. Lesions usually appear as purplish, flat-topped bumps that areoften itchy.Genitals. Lesions on external genitalia resemble those affecting theskin. Lesions affecting the mucous membrane of the vaginaresemble those affecting the mouth.Scalp. When skin lesions appear on the scalp — a rare condition —they may cause temporary or permanent hair loss.Nails. Lichen planus of the toenails or fingernails, also rare, mayresult in ridges on the nails, thinning or splitting of nails, andtemporary or permanent nail loss.
  43. 43. WICKHAM’S STRIAE•Wickham striae are Characteristic, fine, white or greylines or dots seen on the top of the pruritic papularrash of lichen planus•The macroscopic appearance ofthe histologic phenomenon and hypergranulosis, andnamed for Louis Frédéric Wickham.
  44. 44. 1.The Reticular Pattern:•The reticular pattern is considered theclassic form of lichen planus. This formoccurs most frequently, and its white lacylines called Wickhams striae characterizethe lesions• Commonly, the tissue is somewhatraised and the patient may report that theareas in contact with the tongue feelrough in texture
  45. 45. 2.The Plaque Form is represented bythick, plaques of varying sizes, that maybe smooth and appearing somewhatlighter in color than surrounding tissue(non-descriptive leukoplakia)•The tongue is a particular area wherethese plaques may be noticed. They oftenappear as a "bald" area of concern withdenuded papillae.•Higher association with malignanttransformation
  46. 46. 3.The Papular Pattern :•The papular pattern is noted bythe small, pin point papulesappearing as small white dotsmeasuring approximately 0.5 mmin size.• The papular pattern may be inconjunction with other forms oflichen planus and may be overlooked in an oral examinationdepending upon the number ofpapules and the distinctappearance of the lesions
  47. 47. 4.The Bullous Form:•The bullous form consists of vesiclesranging from a few millimeters indiameter to much larger vesicles.•Eating and speaking will usually causethe vesicles to rupture soon afterforming.•This form is seen most often on thebuccal mucosa followed by the tongue
  48. 48. 5.The Atrophic Form- also called erythematous form.•The atrophic form presents as diffuse reddened patches thatmay be in combination with other forms of lichen planus such asthe white striations of the reticular form.•Often found on the attached gingiva, the patient maycomplain of irritation or burning sensations.•Lesions are poorly defined and may have striae at theperipheral areas.• When this form involves the gingiva, it is sometimes referredto as "desquamative gingivitis" which is a clinical term used bysome clinicians denoting a reddened appearance of the tissue.• Other skin diseases may appear similar and this term is notdiagnostic .• In addition, the atrophic form may be confused with BMMPand may appear similar to the gingival depicted
  49. 49. Atrophic Form consists of red, erythematous Atrophic form sometimes described asareas and may include a reticular/plaque like desquamative gingivitis. This form when affecting thesurface. gingiva is definitively diagnosed through biopsy and immunofluorescence. Red marginal area affected by the lichen planus and the noted plaque-like areas superior to the marginal areas.
  50. 50. 6.The Ulcerative Form.•As the term indicates, the ulcerative form features one orseveral ulcers that are often seen in association with any ofthe other disease forms.•This form is quite painful for the patient and the sorenessof the tissue will affect eating and brushing of the teeth.•The ulcerations may affect any tissue surface and mayeven extend into the soft tissues of the tonsillar pillars andesophageals areas.•This form is reported as having a higher number ofmalignant tissue changes.• The ulcerative nature of this form probably allows moredetrimental tissue changes with chronic irritation andpossibly a port of entry for pathogens.
  51. 51. Erosive type of lichen planus with loss of A mixture of erosive and reticular lichensurface epithelium in the buccal mucosa. planus with some candida albicans present on tongue and buccal mucosa. Highly erosive area of lichen planus in the soft plate region
  52. 52. Lichenoid Reactions:•The most common material used in dentistry that may cause alichenoid reaction is amalgam, although other known dental materialshave been implicated as well. The mercury in the metal alloy usuallycauses the hypersensitivity reactions•Hypersensitivity reactions may also be due to flavoring agents or foodproducts or drugs• The lesions are usually isolated and they do not subside as long as thetissue is in contact with the restoration
  53. 53. (iii) HISTOPATHOLOGY•The epidermis is hyperkeratotic with irregular acanthosis and focalthickening in the granular layer.(wedge-shaped hypergranulosis)•The upper dermis has a band like infiltrate of lymphocytic(primarily helper T) and histiocytic cells with many Langerhans cellsat the dermal-epidermal junction.•The lymphocytes are intimately associated with basal keratinocytes, which shows degeneration ,necrosis & resemblance in size &contour to more mature cells of Stratum spinosum. This isSQUAMATIZATION.•As a consequence to this destructive infiltration ,results in redifiningof normal smoothly undulating configuration of dermoepidermaljunction to more angulated zigzag contour ("saw-tooth" appearanceof the rete pegs).•Pigment incontinence due to damage of basal keratinocytes andmelanocytes.(this leads to hyperpigmentation of lesion)
  54. 54. Saw tooth rete pegs
  55. 55. CIVATTE BODIES:•Anucleate ,necrotic basal cells become incorporated into inflamed papillarydermis -these are civatte bodies or colloid bodies.•These are characteristic of lichen planus (but can be found in any chronicdermatitis where basal keratinocytes are injured)•In addition to apoptotic keratinocytes, colloid bodies are composed of globulardeposits of IgM(occasionally immunoglobulin G [IgG] or immunoglobulin A [IgA])and complement.•Max Joseph clefts : Basal keratinocytes weakens the epithelial interface causinghistologic clefts
  56. 56. (iv) MANAGEMENT1.Corticosteroids 3.Nonsteroidal ointmentsCorticosteroids may reduce •Calcineurin inhibitors, which areinflammation associated with oral closely related to or identical to orallichen planus. The side effects vary medications used to prevent rejectiondepending on types of usage of transplanted organs.•Topical. mouthwash or ointment •These treatments appear to be•Oral. taken as a pill effective for the treatment of oral lichen•Injections. Injections may be planus. Examples :Tacrolimus (Protopicadministered directly into lesions. ointment) and pimecrolimus (ElidelRepeated use of corticosteroid cream).injections can cause some of the sameside effects as oral corticosteroids. 4.Addressing triggers Drugs. stop the drug or try an alternative drug2.Retinoids Allergen. advised to avoid the allergenRetinoid are synthetic versions of vitamin Stress. stress may be a factor thatthat can be applied as a topical ointment complicates symptoms or triggers theor taken orally. recurrence
  57. 57. • White sponge nevus (WSN) is a rare autosomal dominant disorder, with a high degree of penetrance and variable expressivity.• The cause is a mutation in the mucosal keratin 4 or keratin 13 genes. Since it is inherited, it can be present at birth or at puberty.• It is a benign, uncommon, and predominantly affects non-keratinized stratified-squamous epithelia
  58. 58. (i) ORAL MANIFESTATIONS•A thick, corrugated bilateral white plaque with aspongy texture, usually on the buccal mucosa, butsometimes on the labial mucosa, alveolar ridge orfloor of the mouth.•Asymptomatic•The gingival margin and dorsum of the tongue arealmost never affected.•Although this condition is perfectly benign, it isoften mistaken for leukoplakia•Ragged white areas which can be removed bygentle rubbing
  59. 59. (ii) HISTOPATHOLOGY•Hyperparakeratosis epithelium , acanthosispresent•Basal layer intact•Cells of spinous layer show Intracellularedema•Submucous have inflammatory cellinfiltrate•Vacuolated and dyskeratotic epithelialkeratinocytes are present that demonstrateperinuclear eosinophilic condensations.
  60. 60. cells of spinous layer showa shrunken nucleus (friedegg appearance) Hyperparakeratosis, acanthosis, and spongiosis in stratified squamous epithelium. The associated connective tissue is usually free of inflammation