A Classical Manifestation of Eosinophilic Granulomatosis with Polyangiitis with an Unusual Catastrophic Outcome

Abstract

A 57-year-old male patient, with a known case of bronchial asthma presented with acute dyspnea, cough with expectoration, fever, severe myalgia, and pedal edema. On physical examination, he had tachypnea, high temperature, bilateral polyphonic rhonchi, audible wheeze, and asymmetric sensory and motor neuropathy. Cutaneous examination revealed multiple palpable purpuric lesions with few hemorrhagic bullae and crusted erosions distributed symmetrically over bilateral limbs suggestive of cutaneous small vessel vasculitis. Investigations showed elevated total counts, eosinophilia, deranged electrolytes, and abnormal liver and renal function tests. Histopathology revealed eosinophilic predominant vasculitis. Testing for perinuclear anti-cytoplasmic antibody was positive. A diagnosis of eosinophilic granulomatosis with polyangiitis (Churg–Strauss disease) was thus confirmed. The patient succumbed in 2 weeks due to multi-organ failure. All the characteristic findings were found in our patient, but a fatal outcome in a very short period after diagnosis was an unusual presentation.

Keywords

• bronchial asthma
• cutaneous small vessel vasculitis
• eosinophilia
• eosinophilic granulomatosis with polyangiitis