, , vulgaris, Epidermolysis bullosa, multiform LICHEN PLANUS

 A chronic mucocutaneous disease of unknown cause  Considered to be immune mediated  Typically presents as bilateral white lesions  A premalignant lesion LICHEN PLANUS

 CLINICAL FEATURES  Older adults  Characteristic white striae known as WHICKMAN’S STRIAE  Clinical types: ○ Reticular ○ Erosive ○ Plaque ○ Papular ○ Erythematous LICHEN PLANUS

 RETICULAR FORM  Interlacing white striae producing an annular or lacy pattern  These are known as whickman’s striae  Buccal mucosa most commonly affected LICHEN PLANUS

 Reticular lichen planus LICHEN PLANUS

 PLAQUE FORM  Resembles clinically but in multifocal areas  Slightly elevated to flat  Dorsum of tongue and buccal mucosa LICHEN PLANUS

 Plaque form of lichen planus LICHEN PLANUS

 ERYTHEMEATOUS / ATROPHIC FORM  Red patches with fine keratotic striae  Patient complains of burning sensation, sensitivity and discomfort  Tongue and attached gingivae LICHEN PLANUS

 Erythemeatous/ atrophic lichen planus LICHEN PLANUS

 EROSIVE FORM  A lesion with central erosive area covered by a pseudo membrane  White keratotic srtiae may be seen around the margins periphery LICHEN PLANUS

 Erosive lichen planus LICHEN PLANUS

 BULLOUS VARIANT  Rare varient composed of vesicles which on rupturing leave painful ulcers  Posterior buccal mucosa LICHEN PLANUS

 Cutaneous lichen planus may be seen in the form of flat topped, small, voilaceous, pruritic lesions LICHEN PLANUS

 Cutaneous lichen planus

LICHEN PLANUS

1. Hyperkeratosis 2. Basal cell degeneration with apoptotic keratinocytes 3. A band of lymphocytic infiltrate beneath the epithelial  Epithelium may be hyperplastic or atrophic  Saw tooth rete ridges are hallmark LICHEN PLANUS

 Ovoid keratotic apoptotic bodies also seen in the basal layer  Theses are called CAVIETTE BODIES LICHEN PLANUS LICHEN PLANUS

 DIFFERENTIAL DIAGNOSIS  Leukoplkia  Liechenoid reactions  Hairy leukoplkia  White sponge  Cheek chewing  LICHEN PLANUS

 TREATMENT  Can not be cured  Corticosteroids  Can not be cured but controlled VESICULO-BULLOUS LESIONS

 Mucous membrane pemphigoid   Erythema multiform  Linear IgA disease VESICULO-BULLOUS LESIONS

 VESICLES:  Superficial 5 mm or less in diameter filled with clear fluid  BULLAE:  Large blisters greater than 5 mm  PUSTULE:  with purulent exudate  :  Lesion with loss of epithelium PEMPHIGUS VULGARIS  Immunolgic disease  Intraepithelial blister formation  is characteristic  Characterized by blister formation, fluid loss, electrolyte imbalance  4 types: 1. Vulgaris 2. Foliaceous 3. Erythematosus 4. Vegetans PEMPHIGUS VULGARIS

 Different types effect epithelium at different levels  Vegetans and vulgaris involve the whole epithelium  These 2 types involve the  Etiology includes circulating against IgG which are reactive against the epithelial tonofilaments PEMPHIGUS VULGARIS  CLINICAL FEATURES  Painful bullae which after rupturing give rise to ulcers  Oral lesions precede cutaneous lesions  Ulcers are red, painful apthous like  Positive NIKOLOSKY SIGN  4th and 5th decade PEMPHIGUS VULGARIS

 HISTOPATHOLOGY  Intraepithelial, suprabasal clefting  Keratinocyte acantholysis  Loss of desmosomal attachment  Broken epithelial cells called TZANK CELLS MUCOUS MEMBRANE PEMPHIGOID  ETIOLOGY:  Autoimmune reaction to basement membrane  CLINICAL FEATURES:  Oral mucosa (gingiva)  Older adults  Ulcers are red with persistant discomfort and pain MUCOUS MEMBRANE PEMPHIGOID  HISTOPATHOLOGY:  Subepithelial clefting  Separation below the epithelium BULLOUS PEMPHIGOID

 Same as mucous membrane pemphigoid, the only difference is that circulating titers are easily detectable in BP  7th—8th decade  more involved than mucosa  Clinically similar to MMP  Attached gingiva, , buccal mucosa and floor of BULLOUS PEMPHIGOID

 HISTOPATHOLOGY:  Subepithelial clefting  Similar to MMP HERPETIFORM

 IgA in tissue  No proper etiology  Young and middle aged with a male prediclation  Papular, erythemeatous, vesicular, pruritic cutaneous lesions  Distributed over extensor surfaces like elbows and sacrum DERMATITIS HERPETIFORM

 Very rare in oral cavity  Vesicles which rupture to form ulcers  Ulcers have a fibrinous base with erythemeatous margins  May involve keratinized and non keratinized areas DERMATITIS HERPETIFORM

 HISTOPATHOLOGY:  Subepithelial clefting  Nutrophils, eiosinophils, fibrin at the papillary  Perivascular lymphogenic inflammation LINEAR IgA DISEASE

 Chronic  Affects gingiva  Ulcerative bullae  HISTOPATHOLOGY:  Seperation at the basement membrane level  Neutrophils and eisinophils fill this gap ERYTHEMA MULTIFORM

 An inflammatory immune mediated mucocutaneous disease  Etiology include previous like herpes, drugs (sulfa), malignancy, crohn’s disease  3 clinical types:  Minor  Chronic minor  Major ERYTHEMA MULTIFORM

 EMMajor has an acute onset seen in young patients  Also known as STEVEN JOHNSON SYNDROME  Large blister formation followed by sloughing of skin  EMMinor involves skin forming foliceous, diffuse or focal areas of erythemeatous lesions ERYTHEMA MULTIFORM  Concentric red areas surrounded by pale peripheral zone  CHRONIC EM mildest form with smaller lesions for shorter duration  HISTOPATHOLOGY:  Intercellular and intracellular edema of overlying epithelium  Vasodilatation of blood vessels along with perivascular inflamation  intraepithelial clefting IMMUNOFLORESCENCE

 2 types: 1. Direct 2. Indirect  DIRECT IMMUNOFLORESCENCE:  Used to detect autoantibodies bound to the patients tissue  The antibodies bind to any site where human immunoglobulin is present IMMUNOFLORESCENCE

 INDIRECT IMMUNOFLORESCENCE:  Used to detect antibodies circulating in pts serum IMMUNOFLORESCENCE

 Pemphigus vulgaris  Positive intercellular  IDIF also positive

 MMP  Positive basement membrane  IDIF negative

 BP  Positive basement membrane  IDIF positive IMMUNOFLORESCENCE

 Erythema multiform  Nondiagnostic DF  IDIF negative

 Linear IgA disease  DIF shows IgA at the basement connective tissue interface