A 15-year-old Caucasian female presented to pediatric rheumatology clinic for evaluation of an intermittent, nonpruritic, painful rash of 2 months duration over her shins and knees. She endorsed fatigue, weight loss, xerostomia, irregular menses, abdominal pain, shoulder and back stiffness, and left calf myalgias. Skin examination was notable for blanching erythematous macules on the bilateral lower extremities. Vital signs and the rest of her examination were unremarkable.Laboratory evaluation revealed a normal complete blood count and differential, comprehensive metabolic panel, coagulation panel, and von Willebrand antigen. Antinuclear antibody, anti-neutrophil cytoplasmic antibody, and cryoglobulin were negative; total complement and urinalysis were normal. Her erythrocyte sedimentation rate was 43 mm/h (0-13), and C-reactive protein was 1.63 mg/dL (<0.8). Radiographic examination and vascular ultrasound of her lower extremities were unremarkable. Biopsy of her rash revealed a perivascular lymphocytic infiltrate.The patient was lost to follow-up and returned 19 months later at which time she reported a 2-month history of back pain. She also developed chest, shoulder, arm, and bilateral lower extremity pain with a different leg rash. She reported a 2.7 kg weight loss, daily emesis, fatigue, and hypertension at her pediatrician's office 2 weeks previously. Manual blood pressure reading was 155/95 mm Hg (>99th percentile for age and height). Weight was 57.6 kg, approximately the 50th percentile. Skin exam was notable for blood-filled, bullous, necrotic lesions on the lower extremities and plantar surfaces with telangiectasias on her thighs (Figure 1). Given her hypertension, she was admitted to the hospital for evaluation.
Hospital CourseAdmission workup was consistent with an inflammatory process and acute kidney injury (Table 1), and renal ultrasound showed bilateral hydroureteronephrosis and a fluid collection above the bladder. Shortly after admission, the patient developed respiratory decompensation and was transferred to the pediatric intensive care unit for closer monitoring. Urology emergently placed bilateral percutaneous ureteral stents, after which the patient's creatinine 660544C PJXXX10.