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3. IT IS A TYPE OF HYPERSENSITIVE REACTION
ORAL BULLAE EROSIONS ,ULCERS AND CRUSTED LIPS
SKIN LESIONS ARISE ON CHEST RATHER THAN EXTREMITIES
IT IS ASSOCIATED WITH MEDICATIONS AND
MYCOPLASMA PNEUMONIAE INFECTION
COMMON DRUGS ARE- SULFONAMIDES, ANTICONVULSANTS
NSAIDS & ALLOPURINOL
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4. THE PRIMARY CYTOKINE INVOLVED IS TUMOR NECROSIS FACTOR
TNF-α
TREATMENT
HIGH DOSES OF SYSTEMIC CORTICOSTEROID
IV IMMUNOGLOBINS
THALIDOMIDE
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5. ETIOLOGY AND PATHOGENESIS
HYPERSENSITIVITY REACTIONS ARE LIKELY CAUSED BY SENSITIZING
CONTACTANT .
BECAUSE OF INTENSE PLASMA CELL INFILTRATION IT IS
BELIEVED THAT THIS IS A B-CELL MEDIATED DISORDER,
WITH T- CELLS AUGMENTING THE RESPONSE.
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6. ORAL HYPERSENSITIVITY REACTIONS MAY TAKE THE FOLLOWING
FORMS:
ACUTE ONSET OF ULCERS
RED AND WHITE RETICULATED LESIONS
FIXED DRUG ERUPTION
MARKED EROSIONS OR ERYTHEMA WITH OR WITHOUT
ULCERATIONS CALLED PLASMA CELL STOMATITIS[PCS]
SWELLING OF THE LIPS /ANGIOEDEMA
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7. CLINICAL FINDINGS
PCS OCCURS WITHIN DAYS OF EXPOSURE TO CONTACTANTS
HOARSENESS , DYSPHAGIA AND MILD AIRWAY OBSTRUCTION
ENDOSCOPY MAY REVEAL ERYTHEMATOUS AND THICKENED
MUCOSA OFTEN WITH A COBBLESTONING PATTERN FROM
THE EDEMA.
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8. ORAL MANIFESTATIONS
PCS PRESENTS AS A BRIGHTLY ERYTHEMATOUS MACULAR AREAS
OF THE ORAL CAVITY , ALMOST ALWAYS INVOLVING THE
MARGINAL AND ATTACHED GINGIVA OR ALVEOLAR MUCOSA
AND OFTEN INVOLVING OTHER SOFT TISSUES , SUCH AS THE
MAXILLARY AND MANDIBULAR SULCUS OR BUCCAL MUCOSA
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9. DIFFERENTIAL DIAGNOSIS
ERYTHEMATOUS / EROSIVE LICHEN PLANUS
AUTOIMMUNE VESICULOBULLOUS DISORDERS
PUBERTAL/PREGNANCY INDUCED GINGIVITIS
ERYTHEMATOUS CANDIDIASIS
THE BIGGEST DIFFFERENCE IS RAPID ONSET OF PCS AND THE
GENERALIZED GINGIVAL INFLAMMATION WITHOUT DESQUAMATION,
ULCERATION OR BLISTERING
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10. LABORATORY FINDINGS
PATCH TESTING TO IDENTIFY THE ALLERGEN
BIOPSY PARAKERATOSIS , EPITHELIAL HYPERPLASIA,
NEUTROPHILIC EXOCYTOSIS , NUMEROUS SPONGIOTIC PUSTULES
DENSE INFILTRATION OF PLASMA CELL IN LAMINA PROPRIA,
MANY DILATED CAPILLARIES LIE CLOSE TO THE SURFACE
IMMUNOPEROXIDASE STAINS POLYCLONAL PLASMA CELL
INFILTRATION
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11. MANAGEMENT
PCS IS SELF LIMITING IF ALLERGEN IF REMOVED
ANALGESICS & ANTIINFLAMMATORY AGENTS
TOPICAL STEROIDS
GINGIVECTOMIES MAY ME NEEDED TO RECONTOUR LESIONS
THAT ARE LONG STANDING AND MORE FIBROTIC
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12. RAS IS A DISORDER CHARECTERIZED BY RECURRING ULCERS
CONFINED TO THE ORAL MUCOSA IN PATIENTS WITH NO
OTHER SIGNS OF DISEASE
RAS IS CONSIDERED AS A DIAGNOSIS OF EXCLUSION SINCE
HEMATOLOGIC DEFICIENCIES , IMMUNE DISORDERS AND
CONNECTIVE TISSUE DISEASES MAY CAUSE LESIONS SIMILAR
TO RAS
RAS AFFECTS APPROX 20% OF GENERAL POPULATION.
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13. ETIOLOGY AND PATHOGENESIS
FACTORS ASSOCIATED WITH RAS
GENETIC FACTORS , HEMATOLOGIC DEFICIENCIES , IMMUNOLOGIC
ABNORMALITIES & LOCAL FACTORS SUCH AS TRAUMA AND
SMOKING
LYMPHOCYTOTOXICITY, ANTIBODY DEPENDENT CELL MEDIATED
CYTOTOXICITY, DEFECTS IN LYPHOCYTE CELL SUBPOPULATION.
HEMATOLOGIC DEFICIENCY LIKE SERUM IRON,FOLATE, OR
VIT B12
ANXIETY , PERIODS OF PSYCHOLOGICAL STRESS , LOCALIZED
TRAUMA, MENSTRUATION, URTI, FOOD ALLERGY.
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14. ORAL FINDINGS
BEGIN MOSTLY DURING THE SECOND DECADE OF LIFE
PRODROMAL BURNING BEFORE THE ULCER ERUPTS
INITIALLY-LOCALIZED AREA OF ERYTHEMA
LESIONS- ROUND , SYMMETRIC AND SHALLOW
COMMON SITES- BUCCAL AND LABIAL MUCOSA
HEALING WITHOUT SCARRING IS USUALLY COMPLETE
MAJOR ULCERS- 1 cm IN DIAMETER AND LAST FOR WEEKS TO
MONTHS EXTREMELY PAINFUL AND INTERFERING WITH
SPEECH AND EATING.
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15. DIFFERENTIAL DIAGNOSIS
VIRAL STOMATITIS , PEMPHIGUS , CONNECTIVE TISSUE DISEASES
DRUG REACTIONS AND DERMATOLOGIC DISORDERS
HISTORY SHOULD DISTINGUISH BLOOD DYSCRASIAS , HIV,
CONNECTIVE TISSUE DISEASES
BIOPSY IS INDICATED WHEN IT IS NECESSARY TO EXCLUDE
OTHER DISEASES LIKE GRANULOMATOUS DISEASE SUCH
AS CROHN DISEASE, SARCOIDOSIS , OR BLISTERING DISEASE
SUCH AS PEMPHIGUS OR PEMPHIGOID.
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16. MANAGEMENT
PROTECTIVE EMOLLIENT SUCH AS ORABASE OR ZILACTIN
PAIN RELIEF IS OBTAINED BY USING TOPICAL DICLOFENAC
IN MORE SEVERE CASES – USE OF HIGH POTENCY TOPICAL
STEROID PREPARATION SUCH AS BETAMETHASONE ,OR
CLOBETASOLINTRALESIONAL STEROIDS CAN BE USED TO TREAT
LARGE INDOLENT MAJOR RAS LESIONS
SYSTEMIC THERAPY-COLCHICINE , PENTOXIFYLLINE , DAPSONE,
SHORT BURSTS OF SYSTEMIC STEROIDS ETC
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17. BD INCLUDES RECURRING ORAL ULCERS , RECURRING GENITAL
ULCERS AND EYE INVOLVEMENT
BD IS NOW UNDERSTOOD TO BE A MULTISYSTEM DISORDER
WITH MANY POSSIBLE MANIFESTATIONS
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18. ETIOLOGY AND PATHOGENESIS
CAUSE IS UNKNOWN
IMMUNE DYSREGULATION , INCLUDING CIRCULATING
IMMUNE COMPLEXES, AUTOIMMUNITY, CYTOKINES AND HEAT
SHOCK PROTEINS, IS A MAJOR FACTOR IN THE PATHOGENESIS
OF BD
HLA-B51 GENOTYPE IS MOST FREQUENTLY LINKED TO BD
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19. CLINICAL MANIFESTATION
HIGHEST INCIDENCE OF BD IS IN YOUNG ADULTS BETWEEN
THE AGES OF 25 AND 40 WITH THE ORAL MUCOSA AS THE
MOST COMMON SITE OF INVOLVEMENT
GENITAL AREA- ULCERS OF SCROTUM AND PENIS IN MALES AND
ULCERS OF LABIA IN FEMALES
EYE LESIONS- UVEITIS, RETINAL VASCULITIS , VASCULAR
OCCLUSION OPTIC ATROPHY AND CONJUCNTIVITIS .
BLINDNESS IS A COMMON COMPLICATION
GENERAL INVOLVEMENT- SKIN LESIONS LIKE ERYTHEMA
NODOSUM, ARTHRITIS, CNS INVOLVEMENT
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20. ORAL FINDINGS
RECURRING ORAL ULCERS APPEAR IN OVER 90% OF PATIENTS
DIFFERENTIAL DIAGNOSIS
DIAGNOSTIC CRITERIA-RECURRENT ORAL ULCERS 3TIMES
WITHIN 12 MONTHS
PLUS- RECURRENT GENITAL ULCERATION
EYE LESIONS
SKIN LESIONS
POSITIVE PATHERGY TEST
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21. MANAGEMENT
AZATHIOPPRINE AND OTHER IMMUNOSUPPRESSIVE DRUGS
COMBINED WITH PREDNISOLONE.
PENTOXIFYLLINE
DAPSONE , COLCHICINE ETC.
AGENTS THAT ARE ACTIVE AGAINST CYTOKINE TNF-α, SUCH AS
INFLIXIMAB AND ETHANERCEPT
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22. PEMPHIGUS INCLUDES A GROUP OF AUTOIMMUNE , POTENTIALLY
LIFE THREATENING DISEASES THAT CAUSES BLISTERS AND EROSIONS
OF THE SKIN AND THE MUCOUS MEMBRANE
ETIOLOGY AND PATHOGENESIS
CAUSE- BINDING OF IgG AUTOANTIBODIES TO DSG3 , A
TRANSMEMBRANE GLYCOPROTEIN ADHESION MOLECULE
PRESENT ON DESMOSOMES
PEMPHIGUS MAY COEXIST WITH OTHER DISEASES LIKE
MYASTHENIA GRAVIS
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23. CLINICAL MANIFESTATIONS
THE CLASSIC LESION OF PEMPHIGUS IS A THIN WALLED
BULLA ARISING ON OTHERWISE NORMAL SKIN OR
MUCOSA
PRESSURE TO A NORMAL AREA RESULTS IN FORMATION
OF A NEW LESION- NIKOLSKY SIGN
ANY MUCOSAL OR SKIN SURFACE MAY BE INVOLVED.
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24. ORAL FINDINGS
CLASSIC BULLA ON A NON INFLAMED BASE.
SHALLOW IRREGULAR ULCERS BECAUSE THE BULLA
RAPIDLY BREAKS.
THIN EPITHELIAL LAYER PEELS AWAY LEAVING A DENUDED
BASE.
MOST COMMONLY SEEN ON BUCCAL MUCOSA
PALATE AND GINGIVA MAY ALSO BE INVOLVED
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25. DIFFERENTIAL DIAGNOSIS
LESIONS OF ACUTE VIRAL INFECTION
ERYTHEMA MULTIFORMI
RAS
PEMPHIGOID , OLP .
PROPER HISTORY SHOULD DISTINGUISH SINCE VIRAL LESIONS
AND EM ARE TOO ACUTE IN NATURE
IN RAS THE INDIVIDUAL ULCERS HEAL AND RECUR WHILE
A PV SPREADS PERIPHERALLY
DESQUAMATIVE GINGIVITIS MAY BE PRESENT WITH PV,
PEMPHIGOID OR OLP- BIOPSY IS REQUIRED TO DISTINGUISH
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26. LAB FINDINGS
BIOPSY
ELECTRON MICROSCOPY- ACANTHOLYSIS , LOSS INTERCELLULAR
SUBSTANCE , WIDENING OF INTERCELLULAR SPACES,
DESTRUCTION OF DESMOSOMES & CELLULAR DEGENERATION.
DIRECT IMMUNOFLORESCENCE- IT SHOWS INTERCELLULAR
DEPOSITION OF IgG .
INDIRECT IMMUNOFLORESCENCE- ANTI KERATINOCYTE
ANTIBODIES ARE SEEN AGAINST THE INTERCELLULAR
SUBSTANCE
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27. TREATMENT
HIGH DOSES OF SYSTEMIC CORTICOSTEROIDS
USUALLY GIVEN IN DOSES OF 1 -2 mg/kg/d
MYCOPHENOLATE- A IMMUNOSUPPRESSIVE DRUG IS FOUND
TO BE EFFECTIVE.
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