Sjögren-Larsson Syndrome (SLS) is a rare and serious genetic disease that affects the central nervous system and skin, significantly impacting the quality of life for both patients and caregivers.
Experts do not know how many people have SLS. In Sweden, where the disease was first identified, SLS is estimated to affect 1 in 250,000 people. Using this figure, we estimate that there are about 1,300 SLS patients in the U.S. and potentially an additional 1,200 in France, Germany, Italy, Spain, and the United Kingdom combined. SLS occurs in patients of all racial and ethnic backgrounds, with males and females equally affected.
Experts do not know how many people have SLS. In Sweden, where the disease was first identified. SLS is estimated to affect 1 in 250,000 people.
estimated SLS patients in the U.S.
Using this figure, Aldeyra estimates that there are about 1,300 SLS patients in the U.S. and potentially an additional 1,200 in France, Germany, Italy, Spain, and the United Kingdom combined.
SLS occurs in patients of all racial and ethnic backgrounds, and males and females are equally affected.
What Are the Symptoms of Sjögren-Larsson Syndrome
People with SLS often suffer from intellectual disability, speech impairments, and seizures. SLS can also affect vision and cause light sensitivity. SLS also can result in stiffness and tightness of the legs, making it difficult to move.
People with SLS have scaly, itchy, thickened skin, called ichthyosis. Healthy skin has a natural moisture barrier that holds water in. Individuals with SLS have problems forming the skin’s natural moisture barrier, which makes their skin lose too much water. SLS patients say that their itchy skin makes them constantly uncomfortable, and they often irritate their skin with a lot of scratching.
What Are the Treatment Options for Sjögren-Larsson Syndrome?
There’s no cure for SLS, so treatment focuses on symptom management. Managing the ichthyosis may require bathing several times every day, wiping off dead skin cells, and applying and rinsing off lotions. These kinds of treatment take several hours each day and only provides temporary relief.
The Potential of Reproxalap for Sjögren-Larsson Syndrome
In patients with SLS, a substance called reactive aldehyde species (RASP) may prevent the skin from forming the natural moisture barrier. We are taking a new approach to treating ichthyosis with a topical (applied to the skin) investigational medicine called reproxalap. Reproxalap is designed to reduce RASP levels, which may help manage the skin symptoms that make SLS patients so uncomfortable.
National Organizations for Rare Disorders 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, including 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.
SLS Network Community A supportive Facebook group providing information to family, friends, patients, and others connected with SLS.
Foundation for Ichthyosis & Related Skin Types, Inc. 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.