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.
abstract: 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 sebaceus, central nervous system malformations, aplasia cutis congenita, limbal dermoid, pigmented nevus) to summarize the unique features of this case and review the two similar cases in the literature.
authors.affiliation: Rady Children's Hospital, San Diego, California, USA.
authors.forename: Joseph
authors.initials: J
authors.lastname: Lam
authors.forename: Magdalene A
authors.initials: MA
authors.lastname: Dohil
authors.forename: Lawrence F
authors.initials: LF
authors.lastname: Eichenfield
authors.forename: Bari B
authors.initials: BB
authors.lastname: Cunningham
mesh_terms: D002490:Central Nervous System / Q000002:abnormalities*; D004476:Ectodermal Dysplasia / Q000473:pathology*; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D017880:Limb Deformities, Congenital / Q000473:pathology*; D008548:Melanosis / Q000151:congenital*; D020752:Neurocutaneous Syndromes / Q000151:congenital*; D009508:Nevus, Pigmented / Q000151:congenital*; D012878:Skin Neoplasms / Q000151:congenital*; D013577:Syndrome