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

• Published in: Advances in medical sciences Vol. 57; no. 1; pp. 169 - 173
• Main Authors: Machaczka, M, Klimkowska, M, Hägglund, H
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier Urban & Partner Sp. z o.o 01.06.2012; Versita
• Subjects: 1-Deoxynojirimycin - therapeutic use; Aged; Gaucher disease; Gaucher Disease - drug therapy; Humans; Inflammation; Leukocytoclastic vasculitis; Male; Medicin och hälsovetenskap; Miglustat; Treatment Outcome; Vasculitis, Leukocytoclastic, Cutaneous - drug therapy; Leukocytoclastic vasculitis; Inflammation; Gaucher disease; Miglustat
• ISSN: 1896-1126; 1898-4002; 1898-4002
• DOI: 10.2478/v10039-012-0021-y

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.