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Year : 1992  |  Volume : 58  |  Issue : 1  |  Page : 52

Biochemical abnormality in sjogren-larsson syndrome


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How to cite this article:
. Biochemical abnormality in sjogren-larsson syndrome.Indian J Dermatol Venereol Leprol 1992;58:52-52

How to cite this URL:
. Biochemical abnormality in sjogren-larsson syndrome. Indian J Dermatol Venereol Leprol [serial online] 1992 [cited 2020 Jun 6 ];58:52-52
Available from: http://www.ijdvl.com/text.asp?1992/58/1/52/3748

Full Text

To the Editor,

This is in reference to the case report published in Ind J Dermatol Venereol Leprol, 1991; 57: 98-99. The report by Mittal et al describes a case of Sjdgren­Larsson syndrome. They state that an abnormality of lipid metabolism is suspected as a cause of the syndrome. I wish to bring it to the notice that Rizzo et al have already reported on the biochemical abnormality that is found in patients with Sjogren­Larsson syndrome. [1],[2] The cultured skin fibroblasts and leukocytes from patients with this syndrome are deficient in the enzyme fatty alcohol : nicotinamide adenine dinucleotide oxidoreductase. The deficiency of this enzyme results in elevated plasma fatty alcohols in most patients with Sjogren­Larsson syndrome. A genetic heterogenicity in the phenotype is suggested by the absence of aberrant lipid metabolism in a minority of the patients with Sjdgren­Larsson syndrome.


1Rizzo WB, Damman AL, Craft DA. Sjogren­Larsson syndrome : Impaired fatty alcohol oxidation in cultured fibroblasts due to deficient fatty alcohal : nicotinamide adenine dinucleotide oxidoreductase activity. J Clin Invest 1988; 81 : 738-44.
2Rizzo WB, Damman AL, Craft DA, et al. Sjogren­Larsson syndrome : inherited defect in the fatty alcohol cycle. J Paediat 1989; 115-: 228-34


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