Sjögren-Larsson Syndrome (SLS) is a rare disease that causes problems with a person’s central nervous system and skin. There is no FDA-approved therapy for treating the skin problems associated with SLS. Current treatments for the skin are time-consuming and not always effective.

What is Sjögren-Larsson Syndrome?

Sjögren-Larsson Syndrome (SLS) is a rare disease that affects the central nervous system and skin. SLS is a genetic (inherited) disease that is present from birth. People with SLS can have intellectual disability, scaly skin, speech problems, stiffening of the leg muscles, and seizures. Having SLS significantly impacts the quality of life for patients and caregivers.



Experts don’t know how many people are born with SLS. In Sweden, where the disease was first described, it is estimated to affect 1 in 250,000 people.



Using this figure, Aldeyra estimates that there are as many as 1,300 SLS patients in the U.S. and 1,200 in France, Germany, Italy, Spain, and the United Kingdom.

SLS has been diagnosed in patients of all racial and ethnic backgrounds, and male and females are equally affected.

Symptoms of Sjögren-Larsson Syndrome

The central nervous system (CNS) symptoms are cognitive developmental impairment, stiffness and tightness of the legs resulting in difficulty moving, and seizures. SLS patients also have eye problems such as glistening white dots in the eye and sensitivity to light. This is because the eyes are part of the CNS.

In addition, people with SLS have scaly, itchy, thickened skin, called ichthyosis. Healthy skin has a natural moisture barrier that holds water in. People with SLS have problems forming the skin’s natural moisture barrier, which results in water loss. SLS patients say that their itchy skin makes them constantly uncomfortable, as it affects most of the body surface. They often irritate their skin with scratching

 Treatment options

There is no cure for Sjögren-Larsson Syndrome. A patient’s caregiving team will treat different symptoms as they appear. There is no FDA-approved therapy specifically for treating the ichthyosis associated with SLS. Managing the ichthyosis can include bathing multiple times daily, sloughing dead skin cells, and applying and removing lotions. These treatment approaches are time consuming. Many patients can spend several hours per day applying lotions and bathing. Even then, these remedies provide only temporary relief and do not fully resolve the symptoms.

Where Aldeyra comes in

In people with SLS, a substance called Reactive Aldehyde Species (RASP) may prevent the skin from forming the natural moisture barrier. Aldeyra is taking a new approach to treating ichthyosis with a topical (applied to the skin) medicine called reproxalap [link to 2B RASP Page], which is designed to reduce RASP levels. This could help prevent the skin symptoms that make SLS patients so uncomfortable.

Helpful resources

    The National Organization for Rare Disorders (NORD), is a non-profit organization dedicated to helping people with rare diseases. Aldeyra and NORD are working to increase awareness of SLS and provide patients with opportunities to connect with each other and to access information about their disease and ongoing efforts related to diagnosis and treatment.
    Through the collaboration with NORD’s RareLaunch® program, NORD provides mentorship, encourages knowledge sharing, promotes networking, and supports the growth of a strong-independent rare disease patient organization.

    The Foundation for Ichthyosis & Related Skin Types, Inc.® (FIRST) is the world’s leading patient advocacy organization dedicated to improving lives and seeking cures for those affected with ichthyosis or a related skin type. FIRST educates, inspires and connects all affected individuals, their families, medical professionals and the general public with regard to these specific sets of skin disorders. FIRST is also the only patient advocacy organization funding research in the field of treatments and cures for ichthyosis.