[Oral DMSO therapy in 3 patients with lipoidproteinosis. Results of long-term therapy].

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Atıf İçin Kopyala

OzkayaBayazit E., Ozarmagan G., Baykal C., Ulug T.

Der Hautarzt; Zeitschrift fur Dermatologie, Venerologie, und verwandte Gebiete, cilt.48, sa.7, ss.477-81, 1997 (SCI-Expanded)

• Yayın Türü: Makale / Tam Makale
• Cilt numarası: 48 Sayı: 7
• Basım Tarihi: 1997
• Doi Numarası: 10.1007/s001050050613
• Dergi Adı: Der Hautarzt; Zeitschrift fur Dermatologie, Venerologie, und verwandte Gebiete
• Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
• Sayfa Sayıları: ss.477-81
• İstanbul Üniversitesi Adresli: Evet

Özet

Lipoid proteinosis is a rare autosomal recessive disorder with a chronic, benign course. There is no generally accepted systemic therapy apart from the experimental oral use of dimethyl sulphoxide (DMSO) and etretinate in two single cases. We treated two sisters and an unrelated man with lipoid proteinosis with longterm oral DMSO (60 mg/kg/d). At the end of an average treatment time of 3 years, DMSO was withdrawn because it produced no beneficial effects with regard to their skin, mucosal lesions or hoarseness. Additionally, one patient showed progression of her disease with worsening hoarseness and onset of dyspnea, requiring surgical removal of vocal cord infiltrates. Three patients with lipoid proteinosis failed to show any beneficial response to long term treatment with DMSO.