Successful Use of Rituximab for Cutaneous Vasculitis

Abstract

A 23-year-old woman presented with recalcitrant purpuric macules and papules on her forearms, legs, and abdomen. At the age of 13 years, she presented to an outside facility with renal failure of unknown etiology requiring hemodialysis followed by living-related kidney transplantation at the age of 14 years. Renal biopsies had not been performed. The patient did well until 1 year later, when she experienced an upper respiratory illness followed by a new petechial rash on her bilateral lower extremities that was thought to be clinically consistent with HenochSchonlein purpura. Although her initial rash resolved without additional therapy, she continued to experience intermittent purpuric eruptions that were associated with abdominal pain, arthritis, and arthralgia. Over the next several years, her rash became persistent, progressed to involve her forearms and abdomen, and was accompanied by hematuria and increases in serum creatinine levels. She had no pulmonary or neurologic symptoms. Her condition did not respond to trials of prednisone (up to 40 mg/d), cyclosporine (100 mg twice daily), azathioprine (100 mg/d), and etanercept (50 mg twice weekly). Mycophenolate mofetil was not tolerated owing to angioedema. When the patient first presented to our clinic in February 2004, she had purpuric macules and papules over her forearms, thighs (Figure1), legs, and abdomen. Medications at that time included prednisone (10 mg/d), cyclosporine (100 mg twice daily), azathioprine (100 mg/d), lisinopril, atenolol, and erythropoietin. A skin biopsy specimen showed leukocytoclastic vasculitis with perivascular C3 deposition but without detectable IgG or IgA on direct immunofluorescence. Subsequent biopsy of her renal allograft showed no evidence of vasculitis or deposition of immunoreactants (data not shown). Significant laboratory test results included the following values: hematocrit, 29% (reference range, 35%-47%); C4, 10.4 mg/dL (reference range, 20-59 mg/dL); C3, 88 mg/dL (reference range, 86-184 mg/dL); rheumatoid factor, 48 IU/mL (reference value, 20 IU/mL); erythrocyte sedimentation rate, 60 mm/h (reference value, 20 mm/h); and creatinine, 1.4 mg/dL (124 μmol/L) (reference value, 1.2 mg/dL [ 106 μmol/L]). Urinalysis revealed microscopic hematuria but no casts. The results of the following laboratory investigations were normal or negative: determination of serum cryoglobulin levels, protein immunofixation electrophoresis, antinuclear antibody and antineutrophil cytoplasmic antibody tests, serologic tests for hepatitis B and C, urinary protein electrophoresis, and stool guaiac tests.