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

• Published in: Journal of the American Academy of Dermatology 2008-05, Vol.58 (5), p.884-888
• Main Authors: Lam, Joseph, MD, Dohil, Magdalene A., MD, Eichenfield, Lawrence F., MD, Cunningham, Bari B., MD
• Format: Article
• Language: English
• 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
• ISSN: 0190-9622; 1097-6787
• DOI: 10.1016/j.jaad.2007.09.029

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.