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Updated: Jun 4, 2026

Ultrasonographic Evaluation of Salivary Glands for Sjogren's Syndrome: Diagnostic and Monitoring Insights
Published on: October 13, 2023
Do you know this syndrome? Sjogren-Larsson syndrome
Marcela Duarte Villela Benez1, Elisa Fontenelle, Brunela Bastos Tozzi
1Central Hospital of the Army, Rio de Janeiro, RJ, Brasil. mabenez@hotmail.com
Abstract:
We report a typical case of Sjogren-Larsson syndrome in a male patient, aged 20. The Sjogren-Larsson syndrome is a neurocutaneous, autosomal recessive and disabling condition, characterized by congenital ichthyosis, spastic paraplegia and mental retardation. It is caused by deficiency of the microsomal enzyme fatty aldehyde dehydrogenase. It has no cure, but most patients survive up to an adult age. Treatment should be multidisciplinary and dermatological therapy aims at relieving the persistent itching and ichthyosis.
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