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Case Reports

Abstract

Atypical Manifestation of Anca-Negative EGPA in a 24-Year-old Woman

A 24-year-old woman with a history of eosinophilic asthma, allergic rhinitis and chronic sinusitis on Dupixent, presented to the hospital with abdominal pain, fever, drenching night sweats and myalgias for 3 weeks. She reported multiple COVID-positive family members. On review of systems, she noted 10-pound unintentional weight loss and intermittent right upper and lower extremity numbness and tingling. On admission, she was noted to have subtle erythematous macules on the bilateral palms and soles. Workup revealed leukocytosis with eosinophilia, positive serology for COVID-19, and elevated ESR as well as splenic and renal infarcts on imaging studies. Extensive infectious and autoimmune workup was negative including serologies for rickettsial disease, parvovirus, p/c-ANCA, and anti-MPO/PR3. Punch biopsy from the left palmar hand revealed a central blood vessel with necrosis and a prominent inflammatory infiltrate comprised almost entirely of eosinophils with formation of a necrotizing granuloma in the mid dermis. Direct immunofluorescent antibody localization was positive for granular perivascular C3. The patient was subsequently diagnosed with eosinophilic granulomatosis with polyangiitis (EGPA). The patient noted marked improvement in her rash and symptoms with oral prednisone and is planned to transition to treatment with mepolizumab. Sixty percent of EGPA patients are ANCA negative, making clinical pathologic correlation critical for diagnosis. ANCA status is associated with clinical phenotype with positive patients more likely to exhibit cardiac involvement, pleural effusion, fever, and livedo and negative patients more likely to present with cutaneous purpura, renal, sinus and neurologic complications.