SLE is an autoimmune disease involving multiple organs, characterized by a vast array of autoantibodies, paicularly antinuclear antibodies (ANAs), in which injury is caused mainly by deposition of immune complexes and binding of antibodies to various cells and tissues. 1997 Revised Criteria for Classification or Systemic Lupus Erythematosus* Criteria Definition 1. Malar Rash Fixed erythema, flat or raised, over the malar eminences, tending to spare the nasolabial folds 2. Discoid rash Erythematous raised patches with adherent keratotic scaling and follicular plugging, atrophic scarring may occure in older lesions 3. Photosensitivity Rash as a result or unusual reaction to sunlight by patient history or physician observation 4. Oral ulcers Oral or nasolpharyngeal ulceration, usually painless, observed by a physician 5. Ahritis Nonerosive ahritis involving, two or more peripheral joints, characterized by tenderness, swelling or effusion 6. Serositis Pleuruitis-convincing history of pleuritic pain or rub heard by a physician or evidence of pleural effusion, orPericarditis-documented by electrocardiogram or rub or evidence of pericardial effusion 7. Renal disorder Persistent proteinuria >0.5 g/dL or >3 if quantitation not performed orCellular casts-may be red blood cell, hemoglobin, granular, tubular, or mixed 8. Neurologic disorder Serizures-in the absence of offending durgs or known metabolic derangements (e.g. uremia, ketoacidosis, or electrolyte imbalance), orPsychosis-in the absence of offending drugs or known metabolic derangement (e.g. uremia, ketoacidosis, or electrolyte imbalance) 9. Hematologic disorder Hemolytic anemia-with reticulocytosis orLeukopenia- <4.0 x 109 cells/L (4000 cells/mm3) total or two or more occasions, orLymphopenia - <1.5 x 109 cells/L (1500 cells/mm3) on two or more occasions, orThrombocytopenia - <100 x 109 cells/L (100 x 103 cells/mm3) in the absence of offending drugs 10. Immunologic disorder Anti-DNA antibody to native DNA in abnormal titer, orAnti-Sm- presence of antibody to Sm nuclear antigen, orPositive finding of antiphospholipid antibodies based on (1) an abdnormal serum level of IgG or IgM Anticardiolipin antibodies.(2) a positive test for lupus anticoagulant using a standard test, or (3) a false-positive serologic test for syphilis known to be positive for at least 6 months and confirmed by negative Treponema pallidum immunobolization or fluorescent treponemal antibody absorption test. 11. Antinuclear antibody An abnormal titer of antinuclear antibody by immunofluorescence or an equivalent assay at any point in time and in the absence of drugs know to be associated with drug-induced lupus syndrome Sturge Weber syndrome: The so-called po wine stain is a special form of nevus flammeus. These lesions tend to grow during childhood, thicken the skin surface, and do not fade with time. Such lesions in the distribution of the trigeminal nerve are associated with the Sturge-Weber syndrome (also called encephalotrigeminal angiomatosis). This uncommon congenital disorder is associated with facial po wine nevi, ipsilateral venous angiomas in the coical leptomeninges, mental retardation, seizures, hemiplegia, and skull radio-opacities. Thus, a large facial telangiectasia in a child with mental deficiency may indicate the presence of additional vascular malformations.