Acquired Angioedema: A Rare Manifestation of Angioimmunoblastic T Cell Lymphoma

The clinical presentation except age of onset is similar in different types of angioedema. A lymphoproliferative disorder like angioimmunoblastic T cell lymphoma (AITL) rarely presents with symptoms of angioedema. We present extremely rare case of elderly male with recurrent tongue swelling, pruritus with normal levels of complements and C1 esterase inhibitor protein featuring as acquired angioedema, a rare manifestation of AITL. Initial response to corticosteroids may be misleading and occurs as a result of immunosuppression of AITL. High index of suspicion may prompt need for histopathological diagnosis of lymph node biopsy. Definitive chemotherapeutic treatment may achieve long term remission.