The Man With the Swollen Tongue

Celia Maia Cruz, Joao Oliveira, Teresa Sequeira, Miguel Ricardo, Luis Magalhaes, Catarina Mendonca


Eosinophilia is defined as an elevation of the eosinophil count above 0.5 10<sup>9</sup>/L and it can be further classified in mild, moderate or severe according to blood eosinophil count. It is important to recognize the combination of eosinophilia and symptoms caused by eosinophils in order to make the correct diagnostic workup, so that distinction between secondary, primary and idiopathic forms can be made. The authors present a case of a 72-year-old man with asthma diagnosed during adulthood. He had a history of diarrhea for the last 9 months already investigated with abdominal imaging and colonoscopy and interpreted as a pancolitis of undetermined etiology. He presented to the emergency department complaining of a swollen tongue in the last 2 weeks. His laboratory studies showed leukocytosis and eosinophilia with a negative HIV antibody test. CT scan of the head and neck revealed diffuse thickening of the intrinsic muscles of the tongue. As for the chest CT, it showed right and left upper lobe patchy opacities. Biopsies of the regions affected (tongue, small intestine and colon) showed abundant infiltration by polymorphonuclear eosinophils. The cytoplasmic anti-neutrophil cytoplasmic antibody (C-ANCA) was elevated and, according to the clinical and laboratory data described before, the patient was diagnosed with Churg-Strauss syndrome (CSS). Treatment with corticosteroids and cyclophosphamide led to complete remission of symptoms and eosinophilia. Clinicians must be aware of eosinophil-associated disease, an entity that refers to a wide spectrum of disorders but in which eosinophils play a central role. Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as CSS, is a rare systemic vasculitis and a better understanding of the disease is still needed.

J Med Cases. 2016;7(12):543-546


Eosinophilia; Churg-Strauss syndrome; Vasculitis

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