Renal tubular acidosis (RTA) may develop in a large population of patients with Sjogren's syndrome (SS), but most of the subjects are asymptomatic. Here, we report a patient with known rheumatoid arthritis and symptoms of xerostomia, xerophthalmia and periodic paralysis. SS should be considered as a cause of RTA. The treatment of the underlying disorder may ameliorate the symptoms.

Sjogren-Larsson syndrome, first described by Sjogren in 1956, and then jointly with Larsson in 1957, is known to occur in different populations throughout the world (Blumel, Watkins, and Eggers, 1958; Richards, 1960; Link and Roldan, 1958; Zaleski, 1962; Heijer and Reed, 1965; Selmanowitz and Porter, 1967). The condition appears to be a clinical and pathological entity characterized by ichthyos...

Background: Association of Addison's disease with connective tissue diseases such as scleroderma and Sjogren have been rarely reported. Anti-centromere antibody (ACA) has been associated with exocrine gland dysfunction in anti-Ro, anti-La negative Sjogren’s syndrome and may be one of the causes of xerostomia in community. The purpose of this article was to introduce a rare case of scleroderma-S...

By Gustavo C Roman MD (Dr. Roman, Director of the Alzheimer Center of Excellence at the Methodist Neurological Institute in Houston, Texas, has no relevant financial relationships to disclose.) Pedro J Ruiz MD (Dr. Ruiz of the California Pacific Medical Center in San Francisco, California, has no relevant financial relationships to disclose.) Originally released June 28, 2006; last updated June...

Sjogren-Larsson syndrome (SLS) is an inherited disorder associated with impaired fatty alcohol oxidation due to deficient activity of fatty alcohol:NAD+ oxidoreductase (FAO). FAO is a complex enzyme which consists of two separate proteins that sequentially catalyze the oxidation of fatty alcohol to fatty aldehyde and fatty acid. To determine which enzymatic component of FAO was deficient in SLS...

Sjögren-Larsson syndrome is an inherited neurocutaneous disorder characterized by ichthyosis, intellectual disability, and spastic diplegia or tetraplegia. Patients have deficient activity of fatty aldehyde dehydrogenase due to mutations in the ALDH3A2 gene, which results in altered lipid composition of their tissues. In this article, the author discusses new information about the biochemical p...