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SYSTEMIC LUPUS ERYTHEMATOSUS DEFINATION • • SYSTAMIC LUPUS ERYTHEMATOSUS IS A DISEASE OF UNKNOWN ETIOLOGY IN WHICH TISSUES AND CELLS ARE DAMAGED BY PATHOGENIC AUTOANTIBODIES AND IMMUNE COMPLEXES 90% OF CASES ARE WOMEN USUSALLY CHIDBEARING AGE BUT CHILDREN, MEN AND ELDERLY PERSON CAN BE AFECTED PRAVELENCE RATE IS APPROX.15 TO 50 CASES PER 100,000 POPULATION SPECTRUM OF DISEASE • ACLE – ACUTE CUTANEOUS LUPUS ERYTHEMATOSUS • SCLE – SUB ACUTE CUTANEOUS LUPUS ERYTHEMATOSUS • CCLE – CHRONIC CUTANEOUS LUPUS ERYTHEMATOSUS PATHOGENESIS • TISSUE DAMAGE CAUSED BY • AUTOANTIBODIES • IMMUNE COMPLEXES • ABNORMAL IMMUNE RESPONSES ARE • 1. POLYCLONAL ANTIGEN SPECIFIC T & B • CELL HYPERREACTIVITY 2. INADEQUATE REGULATION OF HYPERREACTIVITY • ABNORMAL IMMUNE RESPONSES DEPEND UPON INTERACTION BETWEEN • SUSCEPTIBILITY GENES- ACLE - DR2,DR3 SCLE- HLA-B8,DR3 & DEFICIENCES OF C2,C3,C4 DLE - HLA B-7 DR2,DR3,DQ • ENVIRONMENT• 1. UV LIGHT >70 % CASES HAS PHOTOSENSITIVITY • 2. DRUGS - PROCAINAMIDE, HYDRALAZINE, INH, PHENYTOIN, MINOCYCLINE 3.. VIRUSES CMV, EPSTEIN-BARR VIRUSES PATHOGENESIS CLINICAL MANIFESTATION • ACLE – SKIN LESIONS WAX & WANE IN PARALLEL WITH UNDERLYING DISEASE ACTIVITY, NO SCARRING • LOCALISED - SYMETIRCAL ERYTHEMA & EDEMA AT MALAR EMINENCES • GENERALISED - MORBILLIFORM/ EXANTHEMATOUS ERUOTIONS • SCLE - PHOTOSENSITIVITY • ANNULAR ERYTHEMA, PSORIASIFORM , EM. • • EYRTHRODERMA.& NO SCARRING ASSOCOATION WITH RO/SS-A ANTIBODIES & MALIGNANCIES-BREAST,LUNGS,GI,HODGKIN’S DISEASE. AOTOIMMUNE DISEASES CCLE • DISCOID ERYTHEMATOUS PLAQUES WITH ADHERENT • • • • • • • • • SCALE & FOLLICULAR PLUGS-CARPET TACK SIGN HYPERPIGMENTATION AT PERIPHERY, AROPHIC CENTRAL SCARING, TELENGIEACTASIA, HYPOPIGMENTATION, SCARING ALOPECIAS, OCCURS AT SUN EXPOSED AREAS HYPERTROPHIC DLE BULLOUS LESIONS MUCOSAL – 25% OF CASES LUPUS PANNICULITIS AUTOANTIBODIES ASSOCATION ANTIGEN HIGH DISEASE SPECIFICITY FOR SLE ANA - 90% dsDNA - 60% Sm - 25% rRNp - 10% LOW DISEASE SPECIFICITY FOR SLE ssDNA -60% Histones - 50% Ro/SS-A - 25% MOLECULAR SPECIFICITY CLINICAL ASSOCIATION SLE, LE Nephritis Native DNA Ribonucleoprotein Ribosomal P protein CNS LE Denatured DNA Histones Ribonucleoproteins Ribonucleoproteins Risk for SLE in DLE Drug induced SLE SCLE, neonatal LE, SSj SSJ, SCLE RISK FACTORS FOR DEVELOPMENT OF SLE IN PAITENT OF DLE • • • • • • • • • • DIFFUSE NONSARRING ALOPECIA GENERELISED LYMPHADENOPATHY PERIUNGAL NAIL FOLD TELENGIACTASIA RAYNAUD’S PHENOMENON UNEXPLANED ANEMIA LEUCOPENIA FALSE POSITIVE TEST FOR SYPHILS HIGH TITER OF ANA ANTI ssDNA ANTIBODIES ELEVETED ESR ACUTE LE BUTTER FLY LESION ERYTHEMA AT DORSA OF HAND SKIN LESION OF SCLE SCLE DLE DLE DLE LESION AT PINNA DLE LESION CICATRICAL ALOPECIA ORAL LESION HISTOPATHOLOGY IMMUNOHISTOLOGY TREATMENT • LOCAL – • SUN SCREENS • TOPICAL GLUCOCORTICOCOIDS • SYSTAMIC • • • • • • • • • ANTIMALARIALSCHLOROQUIN HYDROXYCHOLROQUIN DAPSONE RETINOIDS CLOFAZIMINE SYSTAMIC GLUCOCORTICOIDS AZATHIOPRINE CYCLOPHOSPHAMIDE
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