Epidermal Nevus Syndrome (ichthyosis hystrix, linear epidermal nevus)
Epidermal Nevus Syndrome is a rare skin condition related to ichthyosis. The term epidermal nevus is a term applied to a variety of congenital (seen at birth) skin lesions characterized by raised thickened patches of skin, or rough bumpy yellow-brown lesions, sometimes accompanied by an underlying redness (erythema). Epidermal nevi may be small and solitary, or may be widespread but confined to one side of the body or widespread and covering both sides of the body. Lesions generally arise on the trunk and limbs; the scalp and face are not usually involved.
Epidermal Nevus Syndrome is a “mosaic” condition so the gene change does not occur in every cell of the body. It does appear to be inherited, although one form of epidermal nevus is a “mosaic” form of Epidermolytic Hyperkeratosis (EHK). If extensive, the children of affected individuals may have generalized EHK.
Inflammatory Linear Epidermal Nevus (ILVEN) is characterized by tiny, red and inflamed lesions that tend to group together in a linear pattern. The lesions of ILVEN may be intensely itchy. The surface of the lesions may look like eczema or psoriasis. ILVEN most often affects one leg and may extend from the buttock to the foot. It may be present at birth, but usually arises during the first 5 years of life. It is somewhat more common in females.
Treatments for epidermal nevi are generally limited to moisturizing lotions and creams. Low potency topical steroids can relieve any associated itching. Topical calcipotriol may reduce the roughness and bumpiness of the lesions. Surgical or laser removal of the lesions may be a choice in some instances.
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