SCALP syndrome: Sebaceous nevus syndrome, CNS malformations, aplasia cutis congenita, limbal dermoid, and pigmented nevus (giant congenital melanocytic nevus) with neurocutaneous melanosis: A distinct syndromic entity

Nevus sebaceus syndrome (SNS) is a constellation of nevus sebaceus with extracutaneous findings, including the ophthalmologic nervous, and musculoskeletal systems. Didymosis aplasticosebacea is a recently described entity consisting of aplasia cutis congenita and nevus sebaceus, implying twin spotti...

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Veröffentlicht in:Journal of the American Academy of Dermatology 2008-05, Vol.58 (5), p.884-888
Hauptverfasser: Lam, Joseph, MD, Dohil, Magdalene A., MD, Eichenfield, Lawrence F., MD, Cunningham, Bari B., MD
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container_title Journal of the American Academy of Dermatology
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creator Lam, Joseph, MD
Dohil, Magdalene A., MD
Eichenfield, Lawrence F., MD
Cunningham, Bari B., MD
description Nevus sebaceus syndrome (SNS) is a constellation of nevus sebaceus with extracutaneous findings, including the ophthalmologic nervous, and musculoskeletal systems. Didymosis aplasticosebacea is a recently described entity consisting of aplasia cutis congenita and nevus sebaceus, implying twin spotting (didymosis). We describe a neonate with a nevus sebaceus on the scalp and a limbal dermoid on her left eye. Contiguous with the nevus sebaceus was a giant congenital melanocytic nevus and numerous areas of membranous aplasia cutis congenita. We propose the acronym SCALP (nevus s ebaceus, c entral nervous system malformations, a plasia cutis congenita, l imbal dermoid, p igmented nevus) to summarize the unique features of this case and review the two similar cases in the literature.
doi_str_mv 10.1016/j.jaad.2007.09.029
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Haemangioma of the skin, of mucosae and of soft tissue</subject><subject>Humans</subject><subject>Infant, Newborn</subject><subject>Limb Deformities, Congenital - pathology</subject><subject>Medical sciences</subject><subject>Melanosis - congenital</subject><subject>Neurocutaneous Syndromes - congenital</subject><subject>Nevus, Pigmented - congenital</subject><subject>Pigmentary diseases of the skin</subject><subject>Skin Neoplasms - congenital</subject><subject>Syndrome</subject><subject>Tumors of the skin and soft tissue. 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Congenital diseases of the skin. Haemangioma of the skin, of mucosae and of soft tissue</topic><topic>Humans</topic><topic>Infant, Newborn</topic><topic>Limb Deformities, Congenital - pathology</topic><topic>Medical sciences</topic><topic>Melanosis - congenital</topic><topic>Neurocutaneous Syndromes - congenital</topic><topic>Nevus, Pigmented - congenital</topic><topic>Pigmentary diseases of the skin</topic><topic>Skin Neoplasms - congenital</topic><topic>Syndrome</topic><topic>Tumors of the skin and soft tissue. Premalignant lesions</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Lam, Joseph, MD</creatorcontrib><creatorcontrib>Dohil, Magdalene A., MD</creatorcontrib><creatorcontrib>Eichenfield, Lawrence F., MD</creatorcontrib><creatorcontrib>Cunningham, Bari B., MD</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Journal of the American Academy of Dermatology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Lam, Joseph, MD</au><au>Dohil, Magdalene A., MD</au><au>Eichenfield, Lawrence F., MD</au><au>Cunningham, Bari B., MD</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>SCALP syndrome: Sebaceous nevus syndrome, CNS malformations, aplasia cutis congenita, limbal dermoid, and pigmented nevus (giant congenital melanocytic nevus) with neurocutaneous melanosis: A distinct syndromic entity</atitle><jtitle>Journal of the American Academy of Dermatology</jtitle><addtitle>J Am Acad Dermatol</addtitle><date>2008-05-01</date><risdate>2008</risdate><volume>58</volume><issue>5</issue><spage>884</spage><epage>888</epage><pages>884-888</pages><issn>0190-9622</issn><eissn>1097-6787</eissn><coden>JAADDB</coden><abstract>Nevus sebaceus syndrome (SNS) is a constellation of nevus sebaceus with extracutaneous findings, including the ophthalmologic nervous, and musculoskeletal systems. 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subjects Biological and medical sciences
Central Nervous System - abnormalities
Dermatology
Ectodermal Dysplasia - pathology
Female
Hereditary diseases of the skin. Congenital diseases of the skin. Haemangioma of the skin, of mucosae and of soft tissue
Humans
Infant, Newborn
Limb Deformities, Congenital - pathology
Medical sciences
Melanosis - congenital
Neurocutaneous Syndromes - congenital
Nevus, Pigmented - congenital
Pigmentary diseases of the skin
Skin Neoplasms - congenital
Syndrome
Tumors of the skin and soft tissue. Premalignant lesions
title SCALP syndrome: Sebaceous nevus syndrome, CNS malformations, aplasia cutis congenita, limbal dermoid, and pigmented nevus (giant congenital melanocytic nevus) with neurocutaneous melanosis: A distinct syndromic entity
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