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Case 18



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Immunology Cases 2005 Case 18 J P is a 63 year old woman who was admitted to CPMC for evaluation of daily fevers to 102 F rash and diffuse arthralgias joint pains Physical examination was notable for BP 160 95 mm Hg and the presence of a tender palpable purpuric rash on her lower extremities Fig 1 She had no lymphadenopathy enlarged lymph nodes or joint swelling Laboratory examination revealed Hct 34 5 low WBC 7 9 X109 l normal Serum creatinine was 2 0 mg dl elevated Serum bilirubin transaminases and alkaline phosphatase liver function tests were all slightly elevated Her ESR erythrocyte sedimentation rate was 74 very high consistent with inflammation A urinalysis revealed no protein minimal microscopic hematuria 3 4 RBC per high power field and occasional WBCs and numerous granular casts A test for Hepatitis B surface antigen was positive The following tests were negative or normal Coombs heterophile agglutinins anti streptolysin 0 ASLO latex fixation ANA anti nuclear antibodies cryoglobulins and SPEP serum protein electrophoresis All bacterial and fungal cultures were negative Skin tests to tuberculin fungal mumps were all non reactive A chest X ray was normal During her hospitalization she developed paresthesias tingling of the first three fingers on the right hand followed by intermittent burning pain in the same distribution She also complained of sciatica which in retrospect she noted intermittently in the prior 6 months A neurological examination was notable for muscular weakness in the L2 3 distribution EMG electromyogram of the median nerve and L2 L3 was consistent with a neuropathy A biopsy of the skin is shown below Fig 2 She was treated with pulse intravenous methylprednisolone 15 mg kg dose IV X 3 days followed by oral prednisone 1 mg kg day and her fever resolved slowly She remained afebrile but her neuropathy persisted and Cytoxan cyclophosphamide was added to her regimen with resultant improvement in her neurological symptoms Fig 1 Anterior tibial



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