Epidermal nevi and epidermal nevus syndromes in pediatric practice

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Abstract

Epidermal nevi are hamartomas of ectodermal origin. Epidermal nevus syndromes are defined as the presence of an epidermal nevus with the involvement of other organ systems. Epidermal nevi are a manifestation of cutaneous mosaicism. Mosaicism may result from postzygotic somatic mutations. Epidermal nevi are usually located along the Blaschko’s lines. There are organoid (sebaceous nevus, comedonic nevus, eccrine nevus) and non-organoid epidermal nevus (keratinocytic epidermal nevus, inflammatory linear verrucous epidermal nevus). Epidermal nevi can be combined with extracutaneous malformations: skeletal anomalies, neurological changes, eye defects, and then it can be related with epidermal nevus syndrome. The presence in a child of large epidermal nevi and nevi located on the face and scalp requires a multidisciplinary approach involving a pediatrician, neurologist, ophthalmologist and geneticist. Currently, several well-defined epidermal nevus syndromes characterized by organoid and non-organoid (keratocytic) nevi have been described: Schimmelpenning’s syndrome, phakomatosis pigmentosa, Becker’s nevus syndrome, comedonal nevus syndrome, angora hairy nevus syndrome, Proteus’ syndrome, and CHILD syndrome (congenital hemidisplasia with ichthiosiform nevus and limb defects). Thus, follow-up of patients with epidermal nevus syndromes should be individualized and include a thorough history, clinical examination, and, if necessary, genetic testing. Typically, children with small, isolated epidermal nevus do not require further evaluation.

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About the authors

Igor A. Gorlanov

St. Petersburg State Pediatric Medical University

Email: gorlanov53@mail.ru

MD, PhD, Dr. Sci. (Med.), Professor, Head, Department of Dermatovenerology

Russian Federation, Saint Petersburg

Larisa M. Leina

St. Petersburg State Pediatric Medical University

Author for correspondence.
Email: larisa.leina@mail.ru

MD, PhD, Assistant Professor, Department of Dermatovenerology

Russian Federation, Saint Petersburg

Irina R. Milyavskaya

St. Petersburg State Pediatric Medical University

Email: imilyavskaya@yandex.ru

MD, PhD, Assistant Professor, Department of Dermatovenerology

Russian Federation, Saint Petersburg

Olga K. Mineeva

St. Petersburg State Pediatric Medical University

Email: o-mine@ya.ru

Dermatovenereologist, Clinic of Dermatovenerology Skin Disease Clinic

Russian Federation, Saint Petersburg

Svetlana Yu. Kulikova

St. Petersburg State Pediatric Medical University

Email: svet22_07@mail.ru

Postgraduate Student, Department of Dermatovenerology

Russian Federation, Saint Petersburg

Kirill V. Artamonov

St. Petersburg State Pediatric Medical University

Email: kvartamonov@mail.ru

MD, PhD, Dr. Sci. (Med.), Assistant Lecturer, Department of Dermatovenerology

Russian Federation, Saint Petersburg

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Supplementary files

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1. Fig. 1. Nevus of the sebaceous glands, a focus without hair, rounded, yellowish in the area of the scalp in an infant

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2. Fig. 2. Nevus of the sebaceous glands, oval, protruding, orange-colored plaque with a granular surface on the scalp

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3. Fig. 3. Epidermal nevus. Hyperkeratotic linear papules along Blaschko’s lines

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4. Fig. 4. Epidermal nevus. Hyperkeratotic linear yellow papules along Blaschko’s lines

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5. Fig. 5. Epidermal nevus on the sole, hyperkeratotic papules located along Blaschko’s lines

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6. Fig. 6. Inflammatory linear verrucous epidermal nevus

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7. Fig. 7. Schimmelpenning syndrome. There is a systematized sebaceous nevus on the body

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8. Fig. 8. Nevus Becker. A large lesion of irregular shape covered with dark hair

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