The association of X-linked ichthyosis with a deficiency of steroidsulfatase was demonstrated biochemically in cultured skin fibroblasts and epidermal cells in recent years [9,2]. A simultaneous deficiency of arylsulfatase C in this inborn error was shown histochemically in skin sections by Koppe et
✦ LIBER ✦
The ‘acid mantle’ of the skin differs in x-linked and autosomal dominant ichthyosis
✍ Scribed by Öhman, Hans; Vahlquist, Anders
- Book ID
- 123339360
- Publisher
- Elsevier Science
- Year
- 1998
- Tongue
- English
- Weight
- 171 KB
- Volume
- 16
- Category
- Article
- ISSN
- 0923-1811
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We have studied 16 men, from 10 unrelated Italian families, affected by steroid suphatase (STS) deficiency, which is the basic defect of X-linked ichthyosis (XLI). The patients' clinical diagnoses were of either isolated ichthyosis or ichthyosis associated with Kallmann syndrome (KS) (hypogonadotrop