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Epidermal nevus syndrome is an umbrella term used to describe epidermal nevi in association with syndromic features. The first well-described syndrome was Schimmelpenning syndrome, but numerous related syndromes were described in subsequent years.
Learn about Epidermal Nevus Syndromes, including symptoms, causes, and treatments. If you or a loved one is affected by this condition, visit NORD to find.
Epidermal naevus syndromes refer to the co-existence of a keratinocytic epidermal naevus or an organoid naevus with other abnormalities in the skin and other organs. They are sometimes called systematised epidermal naevi. They reflect mosaicism, in which there are two distinct cell lines with different genetics and are due to postzygotic mutation.
This topic will review the pathogenesis, clinical presentation, and management of linear epidermal nevus. Nevus sebaceous is discussed separately. (See "Nevus sebaceus and nevus sebaceus syndromes".) EPIDEMIOLOGY. Epidermal nevi occur in approximately 1 to 3 per 1000 live births; males and females tend to be equally affected .
Epidermal nevus syndrome (ENS) is a rare congenitally acquired syndrome, characterized by the presence of epidermal nevi in association with various developmental abnormalities of the skin, eyes, nervous, skeletal, cardiovascular and urogenital systems.
A clinical entity called epidermal nevus syndrome should be more precisely defined and distinguished by clinical, histopathologic, and genetic criteria. In this review, 4 distinct epidermal...
Epidermal nevus syndrome, also known as Feuerstein and Mims syndrome, [1] [2] and Solomon's syndrome [1]: 775 [3] is a rare disease that was first described in 1968 and consists of extensive epidermal nevi with abnormalities of the central nervous system (CNS), skeleton, skin, cardiovascular system, genitourinary system and eyes.
An epidermal nevus (plural: nevi) is an abnormal, noncancerous (benign) patch of skin caused by an overgrowth of cells in the outermost layer of skin (epidermis). Epidermal nevi are typically seen at birth or develop in early childhood. Affected individuals have one or more nevi that vary in size.
Epidermal nevus syndrome (ENS) is a rare congenitally acquired syndrome, characterized by the presence of epidermal nevi in association with various developmental abnormalities of the skin, eyes, nervous, skeletal, cardiovascular and urogenital systems.
In this review and update on epidermal nevus syndrome, we will cover recent genetic discoveries involving epidermal nevi, including nevus sebaceus, keratinocytic epidermal nevus, nevus comedonicus, congenital hemidysplasia with ichthyosiform nevus and limb defects syndrome, phakomatosis pigmentokeratotica, Becker's nevus, porokeratotic adnexal ...