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말초혈액 호산구증다증이 없는 고열을 동반한 Wells 증후군 1예

A Case of Wells’ Syndrome Presented High Fever without Peripheral Eosinophilia

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키워드

주제분류임상병리학

초록

Wells’ syndrome is eosinophilic cellulitis characterized clinically by an acute dermatitis resembling cellulitis. Peripheral eosinophilia is not necessary for the diagnosis but is present in approximately 50% of during active disease. We report a case of Wells’ syndrome which was clinically difficult to diagnose without biopsy. A 26-year-old woman presented with high fever and skin lesion mimicking urticaria migrating in chest, abdomen and extremities. The skin lesion was aggravated with rise in temperature and was evanescent. Well-demarcated swollen and tender erythematous plaques were in ear lobes, neck, chest, abdomen and extremities. Viral markers, blood cultures and other laboratory test results related to infections such as EBV, CMV and Limedisease were negative. FANA, ANCA and ENA were also negative. There was no peripheral eosinophilia. Skin biopsy performed from the volar side of right forearm revealed the infiltration of eosinophils with multifocal collagen degeneration in dermis without vasculitic process, consistent with the early stage of Wells’ syndrome. The patient was treated with intravenous methylprednisolone and antihistamine with remarkable clinical improvement. Wells’ syndrome should be included in the differential diagnosis of any skin lesion mimicking cellulitis even without peripheral eosinophilia. Confirmatory skin biopsy is mandatory for the definite diagnosis of this disease. (Korean J Asthma Allergy Clin Immunol 2008;28:305-308)

Abstract

Wells’ syndrome is eosinophilic cellulitis characterized clinically by an acute dermatitis resembling cellulitis. Peripheral eosinophilia is not necessary for the diagnosis but is present in approximately 50% of during active disease. We report a case of Wells’ syndrome which was clinically difficult to diagnose without biopsy. A 26-year-old woman presented with high fever and skin lesion mimicking urticaria migrating in chest, abdomen and extremities. The skin lesion was aggravated with rise in temperature and was evanescent. Well-demarcated swollen and tender erythematous plaques were in ear lobes, neck, chest, abdomen and extremities. Viral markers, blood cultures and other laboratory test results related to infections such as EBV, CMV and Limedisease were negative. FANA, ANCA and ENA were also negative. There was no peripheral eosinophilia. Skin biopsy performed from the volar side of right forearm revealed the infiltration of eosinophils with multifocal collagen degeneration in dermis without vasculitic process, consistent with the early stage of Wells’ syndrome. The patient was treated with intravenous methylprednisolone and antihistamine with remarkable clinical improvement. Wells’ syndrome should be included in the differential diagnosis of any skin lesion mimicking cellulitis even without peripheral eosinophilia. Confirmatory skin biopsy is mandatory for the definite diagnosis of this disease. (Korean J Asthma Allergy Clin Immunol 2008;28:305-308)

참고문헌 (20)

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  9. Lee HS, "A case of eosinophilic panniculitis associated with paragonimiasis", Korean J Dermatol, 41, pp.1697~1699 (2003)
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  14. Wells GC, "Eosinophilic cellulitis", Br J Dermatol, 100, pp.101~109 (1979)
  15. Peters MS, "Immunofluorescence identification of eosinophil granule major basic protein in the flame figures of Wells' syndrome", Br J Dermatol, 109, pp.141~148 (1983)
  16. Murray D, "Eady RA. Migratory erythema and eosinophilic cellulitis associated with nasopharyngeal carcinoma", J R Soc Med, 74, pp.845~847 (1984)
  17. Moossavi M, "Wells' syndrome: a clinical and histopathologic review of seven cases", Int J Dermatol, 42, pp.62~67 (2003)
  18. Mitchell AJ, "Recurrent granulomatous dermatitis with eosinophilia. Wells' syndrome", Int J Dermatol, 23, pp.198~202 (1984)
  19. Espana A, "Wells' syndrome (eosinophilic cellulitis): correlation between clinical activity, eosinophil levels, eosinophil cation protein and interleukin-5", Br J Dermatol, 140, pp.127~130 (1999)
  20. Schorr WF, "Eosinophilic cellulitis (Wells' syndrome): histologic and clinical features in arthropod bite reactions", J Am Acad Dermatol, 11, pp.1043~1049 (1984)

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