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.
Joseph MX, Jenkins LE, Wechsler ME, Davis LS. Mepolizumab Treatment of Pediatric Eosinophilic Granulomatosis With Polyangiitis. JAMA Dermatol. Published online February 21, 2018. doi:10.1001/jamadermatol.2017.5888
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