J Korean Med Sci.  2005 Aug;20(4):677-679. 10.3346/jkms.2005.20.4.677.

Hypereosinophilia Presenting as Eosinophilic Vasculitis and Multiple Peripheral Artery Occlusions without Organ Involvement

• Kim SH
• Kim TB
• Yun YS
• Shin JI
• Oh IY
• Sir JJ
• Kim KM
• Park HK
• Kang HR
• Chang YS
• Kim YK
• Cho SH
• Song YW
• Choi DC
• Min KU
• Kim YY

• KMID: 1712754
• DOI: http://doi.org/10.3346/jkms.2005.20.4.677

Abstract

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 progressive. 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-alpha was markedly increased. These results suggest that CD40L (a member of TNF-alpha superfamily) could play a role in the inflammatory processes when eosinophil infiltration and activation are observed. We prescribed prednisolone, cyclophosphamide, clopidogrel, cilostazol, beraprost and nifedipine, and she was discharged.

Keyword

Hypereosinophilic Syndrome; Vasculitis; Tumor Necrosis Factor-alpha; CD40 Ligand

MeSH Terms

Adult
Arterial Occlusive Diseases/*diagnosis/etiology
CD40 Ligand/analysis
Cyanosis/etiology
Diagnosis, Differential
Eosinophilia/*diagnosis/etiology
Female
Gangrene/etiology
Humans
Hypereosinophilic Syndrome/blood/complications/*diagnosis
Immunohistochemistry
Peripheral Vascular Diseases/*diagnosis/etiology
Skin/chemistry/pathology
Tumor Necrosis Factor-alpha/metabolism
Vasculitis/*diagnosis/etiology

Figure

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  Fig. 1 Gross photograph: finger tips show gangrenous changes.

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  Fig. 2 Lower extremity CT angiography; occlusions of right popliteal artery, right posterior tibial artery, right peroneal artery, left posterior tibial, left peroneal artery, left distal anterior tibial artery are seen.

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  Fig. 3 Upper extremity CT angiography; multiple stenosis are seen in the upper extremity arteries below wrist level.

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  Fig. 4 Skin biopsy: Immunohistochemistry show CD40L positive eosinophilic infiltration (×400).

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  Fig. 5 Serum TNF-α level compared with healthy controls and other hypereosinophilia without vasculitis.

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