Eosinophilic granulomatosis with polyangiitis (EGPA or Churg-Strauss syndrome) is characterized by asthma, eosinophilia, and systemic vasculitis of multiple organs. We describe a case of rapidly progressive, antineutrophil cytoplasmic antibody (ANCA)-negative EGPA in a teenager that showed improvement after treatment with mepolizumab, an anti–interleukin (IL)-5 monoclonal antibody.
A teenager with asthma reported an eruption on the upper thighs and palms of 1 month’s duration. Additional complaints included right foot numbness and difficulty ambulating. Over the preceding 6 months, the patient had several hospitalizations for an atypical eosinophilic pneumonia, pleural effusion, and pericarditis requiring management with continuous positive airway pressure.