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Adv Med Sci. 2012 Jun 1;57(1):169-73. doi: 10.2478/v10039-012-0021-y.

Unexpected cure from cutaneous leukocytoclastic vasculitis in a patient treated with N-butyldeoxynojirimycin (miglustat) for Gaucher disease.

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1
Division of Hematology, Department of Medicine at Huddinge, Karolinska Institutet, Stockholm, Sweden. maciej.machaczka@ki.se

Abstract

Cutaneous leukocytoclastic vasculitis (CLV) is a necrotizing inflammation of the small vessels in the dermis. We report the case of a Swedish man with an untreated N370S/L444P Gaucher disease who developed CLV at the age of 79 years. The patient has been treated for CLV with topical and oral corticosteroids, moisturizing agents, and periodically with antibiotics for 3 years without improvement. Administration of miglustat (N-butyldeoxynojirimycin; Zavesca®) because of progress of Gaucher disease resulted in a prompt and durable cure of the CLV.

PMID:
22515974
DOI:
10.2478/v10039-012-0021-y
[Indexed for MEDLINE]
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