A 72-year-old male was admitted to the hospital due to chronic rhinosinusitis and recurrent nasal polyps. Additionally, numerous hemorrhagic bullae, palpable purpura, and necrotic maculae on the face and upper and lower limbs were found, as well as oral ulcers (Fig. 1). Red skin lesions on his face had appeared during the last two outbreaks of nasal polyps too. These lesions had disappeared spontaneously after treatment of the nasal polyps by surgery and steroid nasal spray treatment. Furthermore, late-onset treatment-refractory asthma had recently been diagnosed. Moreover, he complained of numbness in his left leg and weight loss of 5 kg in the previous 2 weeks. He denied fever, headache, abdominal pain, diarrhea, hematuria, and arthralgia.
A biopsy of the nasal mucosa revealed eosinophilic infiltration. Further, a skin biopsy demonstrated perivascular eosinophilic infiltrate and necrotizing vasculitis, as well as a granulomatous reaction (Fig. 2). Direct immunofluorescence showed slight deposition of complement component C3 in blood vessels. Laboratory studies demonstrated leukocytosis (20.6 x 109/L) with distinct eosinophilia (4,280 eosinophils/muL, 59% of the total leukocyte count), a high erythrocyte sedimentation rate (52 mm in the first hour), elevated immunoglobulin E (568 kU/L), and a slightly elevated antinuclear antibody level (1: 320 titer). The other blood tests, p-ANCA, c-ANCA, ENA titer, rheumatoid factor, and hepatitis serology, were negative.
Subsequently, EGPA pursuant to the American College of Rheumatology (ACR) 1990 criteria was diagnosed. A chest X-ray and lung function tests, electrocardiography, transthoracic echocardiography (echo), and a stool culture were normal. After ruling out the involvement of other organs, 100 mg per day of oral methylprednisolone (1.5 mg/kg/day) was administered, which was gradually tapered afterwards. Upon observing clinical improvements under methylprednisolone, oral azathioprine at 50 mg daily was administered as an immunosuppressive agent. The dosage was later increased to 150 mg daily. Seven weeks later, the patient showed up in our clinic with complete clinical and laboratory remission (Fig. 3). By then, he was taking methylprednisolone 20 mg daily and azathioprine 150 mg daily (2 mg/kg).