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Ichthyosis, Sjögren Larsson Syndrome

Abstract

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NORD is very grateful to the members of the Medical and Scientific Advisory Board of the Foundation for Ichthyosis & Related Skin Types for assistance in the preparation of this report.

Synonyms of Ichthyosis, Sjögren Larsson Syndrome

Ichthyosis, Spastic Neurologic Disorder, Mental Retardation
SLS

Disorder Subdivisions

No subdivisions found.

General Discussion

Sjögren-Larsson syndrome is an inherited disorder characterized by scaling skin (ichthyosis), mental retardation, speech abnormalities, and spasticity. Affected infants develop various degrees of reddened skin with fine scales soon after birth. After infancy, the skin loses its redness and dark scales often appear on the neck and under the arms. Additionally, larger plate-like thick scales may develop on the lower legs. Developmental delay, speech abnormalities and seizures may accompany skin symptoms. Spasticity in the legs typically impairs motor ability and walking. Many children with this disorder have glistening white dots or degeneration of the pigment in the retina of the eye.

Organizations related to Ichthyosis, Sjögren Larsson Syndrome

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