Facial Edema in an Elderly Man: An Unusual Presentation of Nonepisodic Angioedema with Eosinophilia

Abstract

Nonepisodic angioedema with eosinophilia (NEAE) is a rare allergic disease with a young Japanese and East Asian female predominance. NEAE features transient, nonrecurrent angioedema and peripheral blood eosinophilia without visceral organ involvement. Angioedema in NEAE occurs on the extremities, while the trunk and face are rarely involved. Here, we report a case of NEAE affecting only the face in an 80-year-old Japanese man. He was otherwise healthy and took no medication until the sudden development of angioedema on the face. The extremities and trunk were not involved. Skin biopsy examination revealed eosinophilic infiltration and degranulation between collagen bundles through the entire dermis, and perivascular and perifollicular infiltration of eosinophils and lymphocytes, but no evidence of vasculitis. Peripheral hypereosinophilia and high serum thymus- and activation-regulated chemokine (TARC) level were noted. Visceral organ involvement, parasite infection, and an allergic response were not detected in the patient. Administration of oral corticosteroid improved his symptoms rapidly and dramatically with improvements in the blood eosinophil count and serum TARC level. After the corticosteroid was discontinued, no recurrence was observed for 3 years. Thus, he was diagnosed as having NEAE. It should be noted that angioedema with eosinophilia might occur with an unusual presentation and might develop in elderly patients.

Keywords

• Eosinophilia
• Angioedema with eosinophilia
• Nonepisodic angioedema with eosinophilia
• Thymus- and activation-regulated chemokine