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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Sprache:eng
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Zusammenfassung: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.
ISSN:0190-9622
1097-6787
DOI:10.1016/j.jaad.2007.09.029