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SYSTEMIC LUPUS ERYTHEMATOSUS

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

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SYSTEMIC LUPUS ERYTHEMATOSUS

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  1. SYSTEMIC LUPUSERYTHEMATOSUS

  2. 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

  3. SPECTRUM OF DISEASE

  4. ACLE – ACUTE CUTANEOUS LUPUS ERYTHEMATOSUS • SCLE – SUB ACUTE CUTANEOUS LUPUS ERYTHEMATOSUS • CCLE – CHRONIC CUTANEOUS LUPUS ERYTHEMATOSUS

  5. 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

  6. 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

  7. PATHOGENESIS

  8. 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

  9. 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

  10. AUTOANTIBODIES ASSOCATION

  11. 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

  12. ACUTE LE BUTTER FLY LESION

  13. ERYTHEMA AT DORSA OF HAND

  14. SKIN LESION OF SCLE

  15. SCLE

  16. DLE

  17. DLE

  18. DLE LESION AT PINNA

  19. DLE LESION

  20. CICATRICAL ALOPECIA

  21. ORAL LESION

  22. HISTOPATHOLOGY

  23. IMMUNOHISTOLOGY

  24. TREATMENT • LOCAL – • SUN SCREENS • TOPICAL GLUCOCORTICOCOIDS • SYSTAMIC • ANTIMALARIALS- • CHLOROQUIN • HYDROXYCHOLROQUIN • DAPSONE • RETINOIDS • CLOFAZIMINE • SYSTAMIC GLUCOCORTICOIDS • AZATHIOPRINE • CYCLOPHOSPHAMIDE

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