Abbreviation: NEAE (nonepisodic angioedema with eosinophilia)
Most patients are young adult females from 20 to 40 years of age.
Clinical features:
(1) angioedema, usually localized to the lower extremities
(2) absence of recurrent attacks (nonepisodic), usually limited to one attack
(3) absence of internal organ involvement
(4) variable arthritis
(5) variable urticaria
(6) exclusion of other causes of angioedema
Laboratory findings:
(1) eosinophilia
(2) normal or minimal increase in the serum IgM concentration
(3) variable elevation in LDH
(4) mild elevation in serum IL-5 and tumor necrosis factor alpha
(5) elevated plasma levels of the chemokine TARC/CCL17
A remission may occur spontaneously or after therapy with low dose prednisone or with antihistamines.
