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Year : 1977  |  Volume : 43  |  Issue : 1  |  Page : 43--46

Sjogren Larsson Syndrome


Correspondence Address:
D N Mulay

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Two cases showing essential features of Sjogren Larsson syndrome namely congenital ichthysiform erythrodermia, spastic disorders, mental retardation and speech defect arc presented. There was no history of consanguinity in parents. Macular dystrophy was present in one case. There was no history of convulsions in any of them. Bony age was almost consistent with their chronological agcs. Aminoaciduria was absent in both cases. There was no dental or osscous dysplasia in either case. Hyperteliorism, defective sweating or dermatoglyphy were not present in our cases. Additional findings in one case was presence of diabetes mellitus in the absenceof any family history of diabetes. Tuberculosis in some form or other was present in both cases Although this finding is not of much importance in our country where tuberculosis is commonly prevalent, it cannot be ignored that both patients are non-ambulatory and there is no case of tuberculosis among family members or near contacts.

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Online since 15th March '04
Published by Wolters Kluwer - Medknow