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A 27-year-old Japanese woman presented with a three-week history of swelling of her hands and lower extremities. She had no history of allergic diseases and had taken no medication prior to the development of her symptoms. There were no remarkable physical findings except for edema of her left hand and bilateral lower extremities (Fig. 1A, B ). A white blood cell count was elevated (12,600/mm3) with eosinophilia (absolute eosinophil count, 8800). Liver and renal function tests and creatine phosphokinase (CK) level were normal, except for a slightly increased level of lactate dehydrogenase (287 U/ml; normal value, 106–211). Erythrocyte sedimentation rate was 2 mm/h. Immunoglobulins (IgG, IgA, IgM, IgE) and IgM against human parvovirus B19 were all within the normal range. Serum level of CCL17/thymus and activation-related chemokine (CCL17/TARC) was elevated (12,700 pg/ml [normal value, <450]).
Fig. 1Alleviation of peripheral angioedema by low dose steroid therapy. A 3-week history of swelling of the left hand (A) and bilateral lower extremities (B) was observed at initial presentation. The peripheral edema was alleviated by a short course of low-dose steroid therapy (C, D).
Fig. 1C, D shows the hands and feet of the patient after treatment.
What is the diagnosis?
2. Discussion
2.1 Diagnosis
Non-episodic angioedema associated with eosinophilia (NEAE). A young Asian woman presented with persistent peripheral swelling with eosinophilia in her peripheral blood (Fig. 1A, B).
Angioedema is caused by extravasation of fluids into interstitial space due to disruption of vascular integrity. Episodic angioedema associated with eosinophilia (EAE) was first described by Gleich, which is characterized by recurrent episodes of angioedema, urticaria, fever, weight gain, eosinophilia and elevated serum IgM levels [
]. NEAE affects young females of East-Asian ethnicity, and can last several months and remit spontaneously or can effectively be treated by low-dose corticosteroid therapy. CCL17/TARC is a chemokine that is responsible for the recruitment of T helper type 2 (Th2) cells to affected tissues [
]. Elevated plasma CCL17/TARC levels have been reported in patients with NEAE.
The patient was treated successfully with oral prednisolone 20 mg/day which was tapered off after two weeks (Fig. 1C, D) with normalization of eosinophil counts and serum CCL17/TARC levels. There was no later recurrence of peripheral edema without treatment.
It is considered that NEAE is an important differential diagnosis of peripheral edema occurring in a young woman of Asian-ethnicity.
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