Researchers presented the case report of a patient with antineutrophil cytoplasmic antibody (ANCA)-negative eosinophilic granulomatosis with polyangiitis (EGPA), a subtype of ANCA-associated vasculitis (AAV), complicated by peripheral nerve damage in the journal Medicine.

EGPA is a disease that can have a multisystemic effect on the body. Asthma is often its first symptom, but the actual diagnosis of EGPA often takes some time. Due to the variety of symptoms that patients may present with, treating this condition may be a challenge. 

A 29-year-old man presented with pyrexia (temperature, 38.5°C) and multiple patchy rashes on both lower limbs that were dark red in color; there was neither tenderness nor itching. He also experienced lower limb muscle soreness aggravated by walking and weight bearing. He had symptoms of nausea, anorexia, abdominal pain, and diarrhea. He previously had a history of asthma and bronchiectasis for 2 years. 

Routine blood investigations revealed a white cell count of 15.68 × 109/L, red blood cells of 7.54 × 1012/L, neutrophils of 9.46 × 109/L, eosinophils of 3.79 × 109/L, percentage of eosinophils of 24.2%, and high-sensitivity C-reactive protein levels of 9.79 mg/L. He was diagnosed with eosinophilia and skin and soft tissue infection. He was prescribed treatment and his fever and rashes subsided. 

Read more about AAV patient education 

However, he returned 2 weeks later with similar symptoms. His vital signs were taken, revealing a temperature of 37.8°C, a heart rate of 123 beats/min, a respiratory rate of 22 breaths/min, and a blood pressure of 102/66 mm Hg. As before, the patient had multiple red patchy rashes on his lower extremities. A repeat blood investigation revealed a number of raised biomarkers: percentage of eosinophils (47.7%), C-reactive protein (49.93 mg/L), erythrocyte sedimentation rate of 20.00 mm/h, interleukin 6 (46.7 pg/mL), alanine aminotransferase (97 U/L), gamma-glutamyltransferase (186 U/L), and alkaline phosphatase (152 U/L). He was later found to be negative for proteinase 3-ANCA, myeloperoxidase-ANCA, p-ANCA, and c-ANCA. 

Chest computed tomography (CT) revealed the presence of bilateral bronchiectasis and peripheral inflammation, subpleural infectious lesions in the left lower lobe, and bilateral ground-glass opacities and multiple small nodules. Abdominal CT showed a locally thickened rectum. A colonoscopy was carried out, revealing multiple rectal ulcers; pathology revealed that these were focal eosinophilic infiltrations. A sinus CT scan demonstrated bilateral maxillary sinus and ethmoid sinus inflammation. Electromyography revealed peripheral nerve damage. His past medical history included asthma.

Taking all these findings into consideration, the patient was diagnosed with systemic EGPA with rare peripheral nerve damage. Appropriate treatment was commenced and the patient’s condition improved. He remains on follow-up. 

“In this case, if a dermatopathological examination had been administered and revealed evidence of eosinophilic infiltration, an earlier diagnosis may have been obtained,” the authors concluded. 


Chen Y, Wan Q, Liu B. ANCA-negative eosinophilic granulomatosis with polyangiitis complicated by peripheral nerve damage: a case reportMedicine (Baltimore). Published online August 4, 2023. doi:10.1097/MD.0000000000034450