Lichen planus, Mucous membrane pemphigoid,Pemphigus vulgaris, Epidermolysis bullosa, Erythema 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 leukoplakia 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 HISTOPATHOLOGY 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 nevus Cheek chewing Candidiasis LICHEN PLANUS TREATMENT Can not be cured Corticosteroids Can not be cured but controlled VESICULO-BULLOUS LESIONS Pemphigus vulgaris Mucous membrane pemphigoid Bullous pemphigoid Erythema multiform Linear IgA disease VESICULO-BULLOUS LESIONS VESICLES: Superficial blisters 5 mm or less in diameter filled with clear fluid BULLAE: Large blisters greater than 5 mm PUSTULE: Blister with purulent exudate ULCER: Lesion with loss of epithelium PEMPHIGUS VULGARIS Immunolgic disease Intraepithelial blister formation Acantholysis 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 oral mucosa Etiology includes circulating autoantibodies against IgG which are reactive against the epithelial desmosome 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 autoantibody titers are easily detectable in BP 7th—8th decade Skin more involved than mucosa Clinically similar to MMP Attached gingiva, palate, buccal mucosa and floor of mouth BULLOUS PEMPHIGOID HISTOPATHOLOGY: Subepithelial clefting Similar to MMP DERMATITIS HERPETIFORM IgA antibodies 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 dermis Perivascular lymphogenic inflammation LINEAR IgA DISEASE Chronic autoimmune disease 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 infections 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.