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ulcerative & vesiculobullous disorder-1.pptx
- 1. 1 Presented by Aparajita Tiwari Pg Istyr Ulcerative & Vesiculo Bullous Disorders Guided by Dr Archana Sudheer Dr Amit Kumar Singh Dr Anjali Kumari Dr Kumar Anand Dr Susmit Sneha
- 2. 2 contents Terminologies Classification Single ulcers Recurrent apthousstomatitis Syndromes associated Pathergy test Traumatic ulcer Malignant ulcer Deep fungal infection Tuberculous ulcer Multiple ulcers
- 3. 3 Acute herpetic gingivostomatitis Recurrentherpetic stomatitis Herpes zoster Pemphigus Mucous membrane pemphigoid Bullous pemphigoid Erythema multiforme Stevens – johnson syndrome Toxic epidermal necrolysis Other vesicullobullous lesions – chicken pox herpangina hand foot & mouth disease
- 4. 4 Vesicles – elevatedblisters containing clear fluid less than 1cm. Bullae – elevated blister- like lesion containing clear fluids greater than 1 cm in diameter. Ulcer – are well circumscribed Sometimes Depressed lesions with epithelial defect that is covered by a fibrin clot , resulting in a yellow – white Appearance. Burket`s 12th edition.
- 5. 5 According to clinicalpresentation & aetiological factor Ulcerative conditions without vesicle/bulla formation • Immunologic diseases • Apthous ulcers • Behcet`s disease • Drug reaction • Contact allergies • Traumatic lesions • Traumatic ulcer • Bacterial infection • Syphilis • Tuberculosis • Actinomycosis • noma Ulcerative conditions forms after vesicle/bulla. • Immunologic diseases • Pemphigus vulgaris • Mucous membrane pemphigoid • Bullous pemphigoid • Erythema multiforme • Epidermolysis bullosa • Viral diseases • Herpes simplex infection • Chicken pox • Herpes zoster • Hand foot & mouth disease • herpangina Burket`s 11th edition
- 6. 6 • Deep fungalinfections • Histoplasmosis • Aspergillosis • Blood disorders • Cyclic neutropenia • Agranulocytosis • Leukaemia • Neoplasms • Squamous cell carcinoma • Carcinoma of major salivary gland • Hereditary disease • Epidermolysis bullosa Burket`s 11 th edition
- 7. 7 Single ulcers Recurrent apthousstomatitis – Recurrent apthous stomatitis is an inflammatory condition characterized by painful recurrent ulcerations in oral Cavity with no other sign of systemic disease. Also called canker sores . The term apthous is derived from a greek word aphtha which means ulcerations.
- 8. 8 Predisposing factors - Localfactor Microbial factor Systemic factors Trauma Bacterial : streptococcal Viral : varicella zoster cytomegalo virus Stress Crohn`s disease , celiac disease Ulcerative colitis , HIVinfection, Psychological imbalance Menstrual cycles Syndromes – MAGIC syndrome, Behcet`s disease , reiter syndrome, sweet syndrome.
- 9. 9 Nutritional factor Allergic Iron, folicacid , zinc deficiency & vitamin B 1,B6, B12 deficiency. Food sensitivity , drug allergies
- 10. 10 Pathogenesis – Lymphocyte infiltrationin the epithelium causing erythema ( preulcerative stage) Followed by localised papule Surrounded by a reactive erythematous halo due to inflammatory reaction. The painful papule then ulcerates(ulcerative phase) A pseudo membrane covers the ulcer , which is infiltrated mainly by neutrophils, lymphocytes and plasma cells. Finally there is healing with epithelial regeneration. (healing phase)
- 11. 11 Classification based onthe nature of recurrence • Simple apthous • Complex apthous • Recurrence occurs 2-4 times a year. • The disease activity is almost continuous throughout the year with newer lesions developing as older lesions heal , usually • Complex apthosis is associated with systemic diseases.
- 12. 12 Classification based onthe frequency of reccurence Type A • Type B • Type C • RAS episodes lasting a few days with tolerable pain and few occurences a year. • Painful RAS lasting 3-10 days with reccurence every month. • Chronic painful course with disease activity almost continous throughout the year.
- 13. 13 Clinical features More commonin females Age – second decade All forms are painful. Patients may have prodromal symptoms of tingling or burning before the appearance of the lesions. Lesions are round ,symmetric and shallow with no tissue tags. Floor is covered grey white pseudo membrane and is surrounded by erythematous halo.
- 14. 14 Minor Size - <0.5 cm Shape – oval Number – 1-10 Location – non keratinized mucosa Healing – 10- 14 days without scarring Based on different forms
- 15. 15 Major Periadenitis mucosa necrotica Recurrence Suttons disease Mikuliczscaring aphthae Size - > 1 cm Shape – oval Number – 1-4 Location – non keratinized mucosa Healing – 10-14 days without scarring
- 16. 16 Herpitiform Size - <0.5 cm Shape – oval Number – 10- 100 Location – any intraoral site Healing – 10- 14 days without scarring
- 17. 17 Syndromes associated withapthous like lesion syndromes Reiter syndrome Behcet`s syndrome MAGIC syndrome Sweet syndrome features • Tetrad of non- gonococcal urethritis, arthritis,conjuctivitis & mucocutaneous lesion • Recurrent oral ulceration, recurrent genital ulcerations,eye lesions,skin lesions. • Mouth & genital ulcers with Inflammed cartilage • Recurrent oral ulceration, pyrexia,thrombophlebitis & cutaneous lesions.
- 18. 18 Differential diagnosis For singleapthous ulcer – Traumatic ulcer Tuberculous ulcer Syphilitic ulcer For multiple apthous ulcer – Primary herpetic gingivostomatitis Allergic stomatitis Erythema multiforme Benign mucous membrane pemphigiod Pemphigus
- 19. 19 Investigations- • Serum estimationof vit B12 , Iron & other nutritional deficiency. • Complete blood count • Cytosmear/exfoliative cytology • Pathergy test to rule out behcet`s syndrome. • Pathergy test - pathergy phenomenon is the state of altered tissue reactivity that occurs in response to minor trauma. • Skin/mucosal injury caused by needle prick induces an inflammatory response. • Bluntsd needle is introduced into the dermis making four needle pricks near to each other at an angle of approx 30 degree such that 3-4 mm of needle is buried within the skin . • Papule seen in atleast 2 needle insertion • Pustule seen in atleast 1 needle insertion.
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- 21. 21 Treatment Minor form –first time with very few recurrences avoidance of stimulus like stress Avoidance of food that are hard , salty , spicy, acidic Treatment of anemia if present. Local analgesics 3-4 application topical Tablet vit B12 with folic acid twice daily for 7 days Antiseptic mouthwash ( benzydamine hydrochloride) 3-4 times a Day. Topical application of drugs like Amlexanox apply 3-4 times a day .
- 22. 22 Treatment Minor form –if ulcers are occuring very frequently topical steroids 3-4 times a day ( clobetasol ointment 0.05%, triamcinolone acetonide 0.1% apply 3-4 times a day). Intralesional steroids ( 10- 20 mg insoluble triamcinolone acetonide or 2 mL dexamethasone in dilution with 0.5 mL saline or 2% lidocaine injected to lesion …..injected directly into the lesion 3-4 times /week or two times / week .
- 23. 23 Treatment Major form – Intralesionalsteroids Combination of topical and systemic steroid ( prednisolone 40- 60 mg / day for 5 days) Immunomodulatory drugs – Levimasole – 150 mg bid for 5 days Dapsone – 100 mg / day for 5 days Azathioprine – 50 mg / day for 5 days
- 24. 24 Disease Aetiologic agent Age/ gender Differentia ting factorInvestigation Treatment Traumatic ulcer Trauma from Different source Like teeth ,de nture,acc ident Any age M=F Ulcer is irregular . Injured tissue Tags will be Present along With bleeding Healed once Stimulus is removed Cytosmear Removal of traumatic Factors, pain relieving medications Malignant Ulcer (SSC minor Salivary gland Malignanc y) Multiple factors Older M>F Rolled Margins & Everted edge With hard base (indurated) & Bleed on palpa Tion. Biopsy Radiographs Tumour Markers. Radiotherapy Chemotherapy surgery
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- 26. 26 Disease Aetiologic agent Age/ gender Differentiating factor Investigation Treatment Ulcer mayhave Exophytic growth Around. May involve deeper Into the bone with hard, fixed lymphadenopat hy. Minor salivary gland malignancy commonly seen on palate. Deep fungal Infections Histoplasmosi s Mucormycoss is Blastomycosis Aspwegillosis Older M=F Very similar to malignant ulcer but patient may Not give history of Habit. Biopsy Systemic antifungal Therapy.
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- 28. 28 Diseases Aetiologic factors Age/ Gender Differentiating factor InvestigationTreatment Tubercul ous ulcer Mycoba cterium tubercul osis Older M=F Irregular lesions with ragged Undermined edges, minimal Indurations & with yellowish Granular base. The mucosa surround the ulcer will be oedematous. Cervical lymphadenopathy. Check for other systemic manifestations like weight loss, pulmonary involvement. Monteux test ZN staining Isolation & culture Chest X ray. Anti tubercular Drug therapy.
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- 30. 30 Disease Aetiological agent Age/ gender Differentiating FactorInvestigation Treatment Chancre of syphilis Treponema palladium Painless ulcer with a smooth surface, raised borders , & an indurated base, punched out edge. Microscopic Examination Serological test Penicillin NonHodge kin`s Lymphoma Malignancies of The lymphoid cell Line. Multiple Precipitation Factor like virus, Chemicals, genetic Older M>F Non-Healing deep Ulceration with ill Defined borders in The surrounding mucosa Mainly on palate, Tongue or alveolar Mucosa. Cervical lymphadenopathy is seen. Biopsy complete Blood count Bone marrow Aspiration Renal, liver, chest Examination. Radiotherapy chemotherapy
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- 32. 32 Differential diagnosis ofa case of single ulcer If ulcers are recurrent larger deeper than herpes, may involves multiple sites, oval to round in shape, with history of stress , nutritional deficiency etc. Apthous stomatitis If ulcers are larger deeper than apthous, irregular, with presence Of trauma, sharp cusp Traumatic ulcer If ulcer is of longer duration , undermined edge with weight loss with minimal indurations & with yellowish granular base Tuberculous ulcer Painless ulcer with a smooth surface, raised borders, and an indurated base, punched out edge Syphilitic ulcer Irregular ulcer, with indurated base, everted edge with/without History of tobacco Malignant ulcer/deep fungal Infection.
- 33. 33 Multiple ulcers –having history of vesicles & bulla Herpes simplex infection/ acute herpetic gingivostomatitis – Belongs to herpes viridae family , is a double stranded DNA virus. Caused by HSV1 ( oral & pharyngeal infection) HSV 2 ( genital infections) Primary herpetic stomatitis Recurrent herpetic stomatitis/ secondary herpetic stomatitis /herpis labialis
- 34. 34 Primary herpetic stomatitis Itis the infection in a person who is not exposed to HSV earlier. Transmitted by droplets infection by saliva or direct contact like kissing Incubation period from 2 – 20 days.
- 35. 35 Pathway of HSVinfection • Primary Herpes simplex is the herpes infection in a person who is not exposed to this earlier. • Following infection and local replication at mucosal surfaces, HSV 1 enters sensory nerve endings and is then transported to the nerve ganglia. The virus reaches nerve ganglia ( trigeminal for HSV1 , lumbosacral for HSV2 ) & remains latent there until reactivated.
- 36. 36 Presence of prodromalfeatures fever , irritability, headache, pain upon swallowing,Regional lymphadenopathy Within few days mouth becomes painful & the gingiva becomes inflamed Formation of multiple vesicles, which are clustered together Vesicles ruptured to form shallow ulcers surrounded by an erythematous halo On gingiva this lesion presents as multiple small ulcer seen as acute marginal gingivitis They heal spontaneously within 7-14 days and leave no scar
- 37. 37 Differential diagnosis If ulcersite not specific • Apthous ulcer • Pemphigus • Pemphigoid • Erythema multiforme If lesion confined only to gingiva • Erosive/ atrophic LP • Pemphigus • Pemphigoid • ANUG
- 38. 38 Investigations • Cytosmear/exfoliative cytology • Isolation& tissue culture • Scrappings of lesion , stain with Wright`s, giemsa stain, PAP stain • Demonstrates balloon cells, multinucleated giant cells & intranuclear inclusions bodies. • Isolation of virus in tissue culture, by DNA scappings from lesions.
- 39. 39 Investigation • Detection ofviral DNA • Antibody titres/ serological methods • Most sensitive & accurate method is PCR technique. • Detection of antibody against herpes.
- 40. 40 Treatment To relieve pain Tomaintain proper fluid Balance Mild – moderate cases Analgesics topical / systemic Diphenhydramine hydrochloride 5 mg / mL rinse 3-4 times. Fluid replacement Acyclovir 5% cream local application 3-4 times for 7 days
- 41. 41 • Severe cases• Acyclovir 200 mg – 400 mg 5 times a day for 7 days • Valacyclovir 1g twice for 7 days • Famciclovir 500mg thrice for 7 days.
- 42. 42 Recurrent / secondary/herpeticlabialis Also called cold sore/ fever blister It is recurrence of herpetic lesions in a patient with previous exposure to primary infection due to different activating factors. Multiple vesicles Formation of ulcer
- 43. 43 pathogenesis The viruses, oncethey have been introduced into the body ( primary herpetic infection) Appear to reside dormantly / latent within the regional ganglia When appropriate trigger occurs ( sunlight, trauma, stress) virus reactivates ,replicates in the ganglion And travels along ganglion to the skin / mucosal site The virus travels down the axon to the periphery and infects the epithelial cells adjacent to the cutaneous nerve endings. The virus causes the epithelial cells to enlarge to form multi- nucleated giant cells ( Tzank cells) Causes cells lysis and formation of vesicles.
- 44. 44 Clinical features • Age/gender– adult • Female > male • Site – at the site of primary inoculation • Adjacent area supplied by involved ganglion • On lips • Intraorally – mucosa tightly bound to periosteum • ( hard palate, attached gingiva , alveolar ridge)
- 45. 45 • Differential diagnosis– • Herpes zoster • Angular cheilitis • Erythema multiforme
- 46. 46 Investigation • Detection ofviral DNA • Antibody titres/ serological methods • Most sensitive & accurate method is PCR technique. • Detection of antibody against herpes.
- 47. 47 Investigations • Cytosmear/exfoliative cytology • Isolation& tissue culture • Scrappings of lesion , stain with Wright`s, giemsa stain, PAP stain • Demonstrates balloon cells, multinucleated giant cells & intranuclear inclusions bodies. • Isolation of virus in tissue culture, by DNA scappings from lesions.
- 48. 48 Treatment To relieve pain Tomaintain proper fluid Balance Mild – moderate cases Analgesics topical / systemic Diphenhydramine hydrochloride 5 mg / mL rinse 3-4 times. Fluid replacement Acyclovir 5% cream local application 3-4 times for 7 days
- 49. 49 • Severe cases• Acyclovir 200 mg – 400 mg 5 times a day for 7 days • Valacyclovir 1g twice for 7 days • Famciclovir 500mg thrice for 7 days.
- 50. 50 Herpes zoster/ shingles/ zona • Varicella zoster is the primary infection resulting from the first exogenous exposure of a susceptible individual to a varicella zoster virus. • Herpes zoster occurs as result of reactivation of varicella zoster virus.
- 51. 51 pathogenesis Chicken poxis the varicella zoster infection in a person who is Not exposed earlier Following infection , varicella zoster enters sensory nerve endings ans is then transported By retrograde axonal transport to the neuronal cell bodies along nerve pathway The virus reaches nerve ganglia and remains latent there until reactivated When an appropriate trigger occurs virus reactivates, replicates in the ganglion Travels along axons to skin/ mucosa causing cluster of smaller erythematous papule With rashes , burning sensation and tingling The rashes develops into small vesicle pus crust formation and healing (4-5 weeks) Sometimes pain continues for more than 3-4 months called post herpetic neuralgia
- 52. 52 Clinical course - Pain– boring , pricking, itching , burning, rashes headache, malaise Are present 1-4 days Inflammatory reaction will result in the development of cluster of vesicles on erythematous base (acute phase) Within 3-4 days , vesicles become pustular and ulcerate and crusts after 7-10 days The lesions tend to follow the path of affected nerve and terminate at the midline. After acute phase resolution of the lesion occurs in 2- 3 weeks with scarring , hypo pigmentation or hyper pigmentation. About 15% cases may proceed to chronic phase with neuralgia associated pain Persists longer than 3 months called post herpitic neuralgia.
- 53. 53 If it affects Maxillarydivision The lesions are localized to areas including cheek, lower eyelid, side of nose, upper lid, upper teeth are, mucous membrane of nose, nasopharynx, tonsils, roof of the mouth. Mandibular division It involves the sides of the head, part of the external ear, auditory canal, the lower lip and part of the mucosa of the mouth.
- 54. 54 Oral manifestations • Unilateralinvolvement . • Lesions of oral mucosa extremely painful, unilateral , scattered vesicles/ulcers surrounded by an erythematous zone. • m/c intraoral sites are anterior portion of tongue, soft palate , cheek. • Intraoral complications include- devitalization of teeth, osteoradionecrosis of alveolar bone, exfoliation of the teeth.
- 55. 55 Differential diagnosis • Herpeslabialis • Angular chelitis • Erythema multiforme • Pemphigus • ANUG in case of gingival involvement
- 56. 56 Investigations • Complete hemogram •Cytology – tzank smear test • Biopsy • Virological tests – viral culture isolation & neutralization of virus Serologic tests: complement fixation test immunofluorescent test VZV membrane antigens Molecular methods : PCR Elisa
- 57. 57 Treatment • To relievepain • Topical analgesics – • Lignocaine patch : 5% patch • EMLA cream ( eutectic mixture of lidocaine 2.5% & prilocaine 2.5% • Capsaicin cream – 0.025 – 0.075% • Systemic analgesics – • Paracetamol/acetaminophen 500 mg 3-4 times a day • Ibuprofen 400 mg 3-4 times a day
- 58. 58 • To decreaseviral load • To relieve chronic pain and to prevent chances of post herpetic neuralgia Antiviral therapy – Acyclovir – 800 mg , orally 5 times a day for 7-10 days Valacyclovir – 1000 mg tid for 7 days Famciclovir – 500 mg three times daily Tricyclic antidepressants- Amitriptyline 10 – 25 mg orally at bedtime , increase dosage by 25 mg every 2-4 weeks.
- 59. 59 Pemphigus Is a groupof potentially life threatening , autoimmune, mucocutaneous diseases charecterised by intraepithelial blistering & erosions of the skin and mucous membrane
- 60. 60 Pathogenesis - Presence ofautoantibodies (IgG,IgA) React with desmosomal glycoproteins ( desmoplakin, desmogleins) Which are present on the cell surface of the keratinocyte ( desmosomes causes cell to cell adhesion in the epithelium. The immune system reaction against these glycoproteins causes a loss of cell to cell Adhesion Resulting in the formation of intraepithelial bullae due to acatholysis ( breaking of Stratum spinosum) Since oral epithelium epresses largely Dsg 3 , but skin expresses Dsg 1 as well as Dsg3 Damage to Dsg 3 results in oral lesions at an early stage , while damage to Dsg 1 results In skin lesions 1
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- 62. 62 Possible aetiological factors •1) drugs – penicillamine • Captopril • Enalapril • Penicillins • Cephalosporins • Piroxicam • 2) viruses – herpes viruses 8 • 3) other autoimmune disorders • Rheumatoid arthritis • Myasthenia gravis • Lupus erythematosus • Pernicious anaemia
- 63. 63 Clinical manifestations The classicallesion of pemphigus is a thin walled bulla arising on otherwise normal skin or mucosa. The bulla rapidly breaks but continues to extend peripherally, eventually leaving large areas denuded of skin. The two characteristics sign that are seen -
- 64. 64 sign Nikolsky sign mechanism The applicationof pressure to apparently normal skin in the surrounding area of a vesicle resulting in the removal of upper layer of epidermis and fromation of new lesion. Two types o nikolsky sign are described- 1) Wet – base of the skin is moist , glistening & exudative suggestive of active disease 2) Dry – base of eroded skin is relatively dry suggestive of active disease healing lesion.
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- 66. 66 • Asboe hansensign/ bulla spreading sign • Marginal nikolsky sign • On application of pressure to an intact bulla, the bulla enlarges by extension to an apparently normal surface • Application of lateral pressure to a pre- existing lesion using a cotton tipped swab , tongue depressor leads to lateral extension of bulla.
- 67. 67 Oral manifestations- Age –adults , rarely in childhood Site – buccal mucosa Palate, gingiva. Clinically – lesion begin as the classic bulla, which rapidly break & form shallow irregular painful ulcers. A thin layer of epithelium peels away in an irregular pattern , leaving a denuded base. The edges of the lesion continue to extend peripherally over a period of weeks until they involve large portions of oral mucosa. Gingival lesions are usually comprised severe desquamative or erosive gingivitis.
- 68. 68 Differential diagnosis 1) Mucousmembrane pemphigoid – Pemphigus shows positive Nikolsky and Asboe Hensen sign while absent in pemphigoid It manifest in other mucous like eye, genital etc. 2) Erythema multiforme - pemphigus shows positive nikolsky and asboe hensen sign absent in EM EM target lesion , absent in pemphigus Pemphigus extends central to peripheral . While EM from peripheral to central.
- 69. 69 Investigations Biopsy of perilesionaltissue & histological & immunostaining Examination Assay of serum antibody titres by direct and indirect Immunofluorescence ELISAs for detection of antibodies to desmoglein 1 & 2
- 70. 70 Treatment condition • Very milddisease (Oral lesions) • Moderate cases ( oral pemphigoid + skin lesions) Drug with doses Topical corticosteroids – Fluocinonide – 0.05% 2-3 applications Daily. Clobetasol proprionate 0.05% (2-3 applications per day). Prednisolone doses of 40-60 mg daily.
- 71. 71 More severe cases Ifprednisolone doses Above 100 mg daily are Unresponsive (pulsed intra venous therapy) • Prednisolone doses 60- 100 mg ( 1- 2 mg/kg/day) • Pulsed intravenous therapy with 1 g of methyl prednisolone in 150 mL of dextrose and water, administered over a period of 90 min once daily , on 1- 5 consecutive days, may be considered.
- 72. 72 Mucous membrane pemphigoid Isa rare group of chroni autoimmune disorders characterized by subepithelial blistering producing lesions that primarily affects various mucous membranes of the body.
- 73. 73 pathogenesis Presence of autoantibodies(IgG,IgA,IgM) Bind with hemidesmosomes or basement membrane gllycoproteins, which will act as self antigen . The immune reaction against these glycoproteins causes detachment of the basal cells from the basement membrane zones Resulting in the formation of subepithelial bullae
- 74. 74 Oral lesions The orallesions may begin as classic bulla which rapidly break & form shallow irregular painful ulcers. • Nikolsky & Asboe Hensen sign – mostly negative • Ocular lesions – • Chronic conjuctivitis with symptoms of burning, irritation , photophobia and excess tearing. • Vesicles & ulcerations is seen only in advances stage. • Scarring following repeated fibrosis can lead to the fusion of the bulbar and palpebral conjuctivae(symblepharon) or the superior & inferior palpebrae (Ankyoblepharon)
- 75. 75 • The conjuctivamay contract and invert the eyelid margin(Entropion),leading to inversion of eyelashes onto the corneal surface with subsequent irritation ( trichiasis).
- 76. 76 Differential diagnoses Pemphigus Erythema multiforme Erosive/ Bullous lichen planus Investigations – Biopsy specimens with histological Direct immunofluorescent techniques
- 77. 77 Treatment condition • Very milddisease (Oral lesions) • Moderate cases ( oral pemphigoid + skin lesions) Drug with doses Topical corticosteroids – Fluocinonide – 0.05% 2-3 applications Daily. Clobetasol proprionate 0.05% (2-3 applications per day). Prednisolone doses of 40-60 mg daily.
- 78. 78 More severe cases Ifprednisolone doses Above 100 mg daily are Unresponsive (pulsed intra venous therapy) • Prednisolone doses 60- 100 mg ( 1- 2 mg/kg/day) • Pulsed intravenous therapy with 1 g of methyl prednisolone in 150 mL of dextrose and water, administered over a period of 90 min once daily , on 1- 5 consecutive days, may be considered.
- 79. 79 Bullous pemphigoid It isquite similar to mucous membrane pemphigoid except – Auto antibodies is against basement membrane zone laminin and so called bullous pemphigoid antigens 230 (BP230) and 180 (BP180) which are found in hemidesmosomes and in the lamina lucida of basement membrane.
- 80. 80 Erythema multiforme It isan acute , Usually self limiting mucocutaneous disorder characterized by unique iris/target lesions and present multiform lesions like macules, papules , vesicles and bullae. It is considerd as autoimmune , psychosomatic disorders, vesiculobullous and type IV hypersensitivity reaction to drugs or food products.
- 81. 81 Aetiology 1) Drugs –sulphonamides, cephalosporins, aminopenicillins, barbiturates,anticonvulsants,allopurinol. 2) Micro organism- Viral agents- hsv1,2 , Epstein barr virus, varicella zoster virus, adeno virus, entero viruses, hepatitis viruses, influenza virus. Bacterial agents- mycoplasma, cornybacterium,neisseria, mycobacterium Fungal agents – histoplasma capsulatum, coccidioidesimmitis Parasites- Trichomonas , toxoplasma gondi. 3) Immune conditions – Hepatitis b immunization Sarcoidosis Graft versus host disease Systemic lupus erythematosus
- 82. 82 Pathogenesis Presence of cellantigen ( viral DNA fragment , drug , metabolites etc. Stimulation of CD4 T cells Production of pro inflammatory mediators like interferon gamma and tumour necrosis Factor alpha Interferon gamma and TNF stimulates the production of additional cytokines and Chemokines Cytotoxic T cells and natural killer cells These cytotoxic T cells , NK cells or chemokines can all induce epithelial damage So erythema multiforme appears to be the result of a cell – mediated immune reaction to the precipitating agent
- 83. 83 Variants Erythema multiforme minor Erythemamultiforme major - Stevens johnson syndrome - Toxic epidermal necrolysis - Oral manifestations – - Oral manifestation of EM minor is less than EM major - Oral lesions may be found on lips , labial mucosa, floor of mouth , soft palate , buccal , gingival mucosa - Clinical stages – the development of oral lesions can be divided into 5 stages :- Macular , vesicular , sloughing ,pseudomembranous And healing
- 84. 84 Lips are extensivelyinvolved with crust formation Dysphagia if lesions are present on oropharynx Presence of enlarged lymph nodes. 1) Steven johnson syndrome – More severe than EM minor and major Involves multiple mucous membranes – oral cavity , the genital , ocular , laryngeal and oesophageal mucosa. Skin – epidermal attachment > 10 % with atypical flat target lesion Systemic involvement is also seen. Mucosal vesicles and bullae occur which ruptures and leave surface Covered with thick white or yellow exudates. Lips may exhibit bloody crusting which are extremely painful.
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- 86. 86 2) Toxic epidermalnecrolysis (Lyell`s disease) More severe and lethal than steven johnsaon syndrome Skin – epithelial attachment is 30% with atypical flat target lesion Systemic involvement is also seen.most common aetiology is drugs Involvement of - mucous membranes oral cavity , genital , ocular , laryngeal and oesophageal and oesophageal mucosae.
- 87. 87 . Typical targets Raised atypical targets Is definedas individual lesion less then 3 cm in diameter Regular round shaped, well defined border , two concentric palpable Oedematous rings , paler than the centre. Similar to target lesions and is palpable erythematous lesions with a rounded shape but poorly defined borders and a dark central area which may erode and become necrotic. These lesions are more common in EM minor. Common in EM major And SJS. Different target lesion in different variety
- 88. 88 Flat atypical targets Flatatypical targets – as their name suggests are not palpable And they form ill- defined erythematous areas with a tendency To central blister formation. These lesions are most common In SJS.
- 89. 89 Multiple targe lesionson hand & leg
- 90. 90 Invetigations for allforms of EM Erythema multiforme is diagnosed clinically. In patients who have target lesions with a preceding or coexisting HSV infection , the diagnosis can be made easily. immunofluorescence can be useful in some cases. Differential diagnosis for oral lesions for all forms of EM Primary herpetic stomatitis Pemphigus Mucous membrane pemphigoid
- 91. 91 Treatment • Mild formsMild cases heal in 2-6 weeks Local wound care Topical analgesics Diphenhydramine hydrochloride syrup mixed with equal amount of antacid liquid in a swish and swallow method 3-4 times daily. Topical steroids – clobetasol Proprionate 0.05%, triamcinolone acetonide – 0.1% - apply 3-4 times a Day.
- 92. 92 For more severecases (for EM major & SJS) If patient is resistant unresponsive or allergic to steroid In cases suspecting herpes suspecting herpes associated EM Systemic corticosteroids Prednisolone 60 – 100 mg daily ( 1- 2 mg / kg /day) Immunosuppressive drugs / steroid sparing drugs . Azathioprine, cyclophosphamide , cyclosporine , methotrexate Acyclovir ( 400 mg twice / day) Valacyclovir (500-1000mg/day) Famciclovir (125-250mg/day)
- 93. 93 disease Aetiological agent Age/ gender Differentiating factorinvestigation treatment Chicken pox Varicella zoster M=F Vesicles with surrounding erythema, rupture soon after Formation & form small eroded ulcers with a red margin Cytosmear isolation Tissue culture Detection of viral DNA Vaccination Fluid replacement Self healing herpangina Coxsack ie A1- 6,A8,A1 0,A22 , B 3 M=F Young adult , child Lesion site specific , limited to The anterior pillars , soft palate , uvula & Tonsils. cytosmear Symptomatic Treatment, self healing Within a weak Other vesiculobullous lesions
- 94. 94 Oral lesion chickenpox herpangina
- 95. 95 Hand foot Mouth disease Coxsackie A5,A9, B2, B5 Child Young Adult M=F Characterized similar to primary herpetic Gingivostomatiti s,ie small vesicles that rapidly Rupture , leaving painful shallow ulcers surrounded by a red halo, but ulcers are not clustered cytosmear Symtomatic treatment Self healing within A week
- 96. 96 Hereditar y Epiderm olysis bullosa Heredi tary Disord er Affects differe nt Genes of epithel ial cytosk eleton Sinc e birt h F> M Bullae and vesicles forms Onthe skin & mucous Membrane which are recurrent & at the site of mild pressure & trauma Biopsy, Immunofluores cence Systemic antibiotic Sytemic steroids
- 97. 97 Multiple ulcers whichdoes not have vesicles or bulla disease Aetiological factor age Differentiating factor investigation treatment Allergic stomatitis Type I , IV Allergic Rection To restoration Toothpaste etc Any age Desquamation Of superficial skin Multiple ulcers. Skin patch test Removal of causati Agent Antihistaminic Mouthwash. Cyclic neutropenia Autosomal Dominant Mutation Of Gene For Neutrohilic elastase Infants Young children Ulcers without Erythematous halo Persists 10-14 days Heals with scaring Complete blood count Bone marrow aspiration Supportive care, Granulocyte colony stimulating factor
- 98. 98 Contact allergic stomatitis Cyclicneutropenia
- 99. 99 Multiple ulcers whichdoes not have vesicles or bulla disease aetiological age Diff erentiating factor investigation treatment Agranulo cytosis Drugs Or infection F>m Ulcers covered by grayish White pseudomembrane Complete blood count. Bone marrow aspiration Supportive care Antibiotics, granulocyte Colony stimulating factor leukaemia Genetic Infection Drugs radiation M>f Deep punched out Ulcer Complete blood count Bone marrow aspiration chemotherapy
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