INTRODUCTION Hypereosinophilia is an abnormal condition defined by the presence of more than 500 eosinophils per microliter of
blood. Hypereosinophilia occurs in allergic reactions, helmi - nthic infestation, collagen vascular disease and malignancies
etc. (1, 2). The diagnosis of the idiopathic hypereosinophilic
syndrome is based on exclusion of other causes of hyper - eosinophilia. That is a disease characterized by unexplained
hypereosinophilia persisting for at least 6 months and lead-
ing to organ damage (3). The most common cutaneous manifestations in idiopathic hypereosinophilic syndrome are either erythematous, pruritic
papules and nodules or angio-edematous and urticarial plaques.
In previous reports, cases with cutaneous vasculitis including
eosinophilic infiltration involving small dermal vessel were
described (4, 5). Eosinophilic vascular inflammation of those
cases showed good response to glucocorticoid treatment. We report here a case with hypereosinophilia and peripheral artery occlusion. It differs from cutaneous eosinophilic vas-
culitis and particularly has no internal organ involvement.
This case has many differences from typical idiopathic hyper-
eosinophilic syndrome. CASE REPORT A 32-yr-old Korean woman presented with lower extremity swelling and pain. One year and three months ago, she developed swelling and itching sense in both lower extremities. She visited an
orthopedic clinic and was prescribed antibiotics with the
impression of having a cellulitis for 1 month. But, this swelling
progressed to her both hands and pain was developed. Bluish
discoloration then appeared at both hands and legs together
with necrosis at some areas. Laboratory investigations revealed
a persistent hypereosinophilia (9,374-16,358/ L). Lower
extremity CT angiography showed that multiple lower ex - tremity arteries were occlusive. A biopsy specimen from her
foot showed perivascular and periadnexal dense eosinophilic
infiltration in dermis and subcutaneous adipose tissue. Bone
marrow finding showed reactive eosinophilia. We performed
microscopic stool exam, ELISA for parasitic antibody, skin
prick test for common allergens and bronchial provocation
test. She had no evidence of parasitic, allergy, connective tis-
sue disease or other known causes of eosinophilia. She was
prescribed oral prednisolone 60 mg, aspirin 100 mg, clopi-
dogrel 75 mg, and beraprost (PGI) 60 mg daily. Her skin Sung-Hwan Kim, Tae-Bum Kim,
Young-Sun Yun, Jung-Im Shin,
Il-Young Oh, Jung-Ju Sir,
Kyung-Mook Kim, Hye-Kyung Park,
Hye-Ryun Kang, Yoon-Seok Chang,
Yoon-Keun Kim, Sang-Heon Cho,
Yeong-Wook Song, Dong-Chul Choi * , Kyung-Up Min, You-Young Kim Department of Internal Medicine, Seoul National
University College of Medicine, Seoul; Department of
Internal Medicine*, Sungkyunkwan University College
of Medicine, Seoul, Korea Address for correspondence Sang-Heon Cho, M.D.
Department of Internal Medicine, Seoul National
University College of Medicine, 28 Yongon-dong,
Jongno-gu, Seoul 110-744, Korea
Tel : +82.2-760-3291, Fax : +82.2-742-2912
E-mail : shcho@plaza.snu.ac.kr 677 J Korean Med Sci 2005; 20: 677-9
ISSN 1011-8934 Copyright The Korean Academy of Medical Sciences Hypereosinophilia Presenting as Eosinophilic Vasculitis and Multiple
Peripheral Artery Occlusions without Organ Involvement We report here a case with hypereosinophilia and peripheral artery occlusion. A
32-yr-old Korean woman presented to us with lower extremity swelling and pain.
Angiography revealed that multiple lower extremity arteries were occlusive. The
biopsy specimen showed perivascular and periadnexal dense eosinophilic infiltration
in dermis and subcutaneous adipose tissue. Laboratory investigations revealed a
persistent hypereosinophilia. She was prescribed prednisolone 60 mg daily. Her skin
lesion and pain were improved and the eosinophil count was dramatically decreased.
After discharge, eosinophil count gradually increased again. Cyanosis and pain of
her fingers recurred. She had been treated with cyclophosphamide pulse therapy.
Her eosinophilia was decreased, but the cyanosis and tingling sense were progres-
sive. The extremity arterial stenoses were slightly progressed. Skin biopsy showed
perivascular eosinophilic infiltration in the dermis and CD40 ligand (CD40L) positive
eosinophilic infiltration. The serum TNF- was markedly increased. These results
suggest that CD40L (a member of TNF- superfamily) could play a role in the inflam-
matory processes when eosinophil infiltration and activation are observed. We pre-
scribed prednisolone, cyclophosphamide, clopidogrel, cilostazol, beraprost and
nifedipine, and she was discharged. Key Words : Hypereosinophilic Syndrome; Vasculitis; Tumor Necrosis Factor-alpha; CD40 Ligand Received : 14 June 2004 Accepted : 2 August 2004 lesion and pain were improved and the eosinophil count was
dramatically decreased (12-92/ L). One month later after
discharge, eosinophil count gradually increased up to 3,465/ L again. Six months later after her discharge, cyanosis and pain of her fingers recurred. A Doppler scan on the extremities
was carried, which showed no significant interval change. She
was treated with cyclophosphamide pulse therapy five times.
Her eosinophilia was then decreased to about 1,000/ L, but
the cyanosis and tingling sense were progressive. She received
a sympathectomy procedure by the thoracoscopic approach
in order to relieve her tingling sense. She then visited our hospital and received re-evaluation. Her fingertips were gangrenous (Fig. 1). CT angiography on
the upper extremity and lower extremity was done. It showed that the lower extremity arterial stenosis slightly progressed
compared with previous study and upper extremity arteries
below the wrist level had multiple stenosis (Fig. 2, 3). The
cerebral and carotid arteries were intact. Liver sonograpy, chest 678 S.-H. Kim, T.-B. Kim, Y.-S. Yun, et al. Fig. 1. Gross photograph: finger tips show gangrenous changes. Fig. 3. Upper extremity CT angiography; multiple stenosis are seen in the upper extremity arteries below wrist level. Fig. 4. Skin biopsy: Immunohistochemistry show CD40L positive eosinophilic infiltration (