Linear epidermal nevus syndrome

• Linear Epidermal Nevus Syndrome (LENS) is a rare genetic skin disorder characterized by the presence of linear, brown or flesh-colored skin patches that are frequently accompanied by neurological, skeletal, or ocular anomalies.

Pathophysiology

• The patches can appear anywhere on the body, but the torso and limbs are the most common locations. The term "linear" in the name of the syndrome derives from the arrangement of the patches in a linear fashion.

Cause

• The exact cause of LENS is unknown, but it is believed to result from a prenatal genetic mutation. Typically, LENS is present at birth, but the patches may not manifest until later in life. During periods of rapid growth, such as during childhood, the patches may become more noticeable.

Prognosis

• LENS is not a dangerous condition, but affected individuals may experience cosmetic and psychosocial issues.

Treatment

• There is no cure for the syndrome, but topical or oral medications, such as corticosteroids or calcineurin inhibitors, can be used to treat the patches.
• In certain situations, surgical removal of the patches may be required.

Summary

• Linear Epidermal Nevus Syndrome (LENS) is a rare genetic skin disorder characterized by linear skin patches that are frequently accompanied by other anomalies. Although the precise cause of LENS is unknown, it is believed to be the result of a genetic mutation. LENS can cause cosmetic issues, but it is not a dangerous condition and can in some cases be treated with topical or oral medications or surgery.

Linear epidermal nevus syndrome Resources
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