BMJ Case Rep. 2026 Sep 16;19(9):e273616. doi: 10.1136/bcr-2026-273616.
ABSTRACT
Eosinophilic granulomatosis with polyangiitis (EGPA) and hypereosinophilic syndrome (HES) share overlapping clinical features, making early distinction difficult, especially in antineutrophil cytoplasmic antibody (ANCA)-negative disease where eosinophil-mediated injury may precede vasculitis. We report a male patient in his mid-20s who initially presented with fever, purpuric rash, neuropathy, marked eosinophilia, hepatic dysfunction and extensive multisystem thrombosis. Biopsy revealed eosinophilic infiltration without vasculitis, supporting a diagnosis of HES. He achieved remission on corticosteroids and anticoagulation but relapsed 5 years later with recurrent thrombosis, purpura, eosinophilia and biopsy-proven eosinophilic vasculitis, prompting reclassification as ANCA-negative EGPA. Treatment with corticosteroids, rituximab and anticoagulation resulted in sustained remission over 36 months. This case highlights the diagnostic continuum between HES and EGPA and the need for long-term follow-up with reassessment of diagnosis. Early recognition of evolving vasculitis is essential to guide appropriate immunosuppressive therapy.
PMID:42749346 | DOI:10.1136/bcr-2026-273616