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 CHID-BEARING 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 MOLECULAR SPECIFICITY CLINICAL ASSOCIATION HIGH DISEASE SPECIFICITY FOR SLE ANA - 90% dsDNA - 60% Sm - 25% rRNp - 10% - Native DNA Ribonucleoprotein Ribosomal P protein SLE, LE Nephritis CNS LE LOW DISEASE SPECIFICITY FOR SLE ssDNA -60% Histones - 50% Ro/SS-A - 25% La/Ss-B - 25% Denatured DNA Histones 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 – SYSTAMIC SUN SCREENS TOPICAL GLUCOCORTICOCOIDS ANTIMALARIALS- CHLOROQUIN HYDROXYCHOLROQUIN DAPSONE RETINOIDS CLOFAZIMINE SYSTAMIC GLUCOCORTICOIDS AZATHIOPRINE CYCLOPHOSPHAMIDE