Linear nevus sebaceus syndrome with right megalencephaly. Case report

A. Patrizi, D. Pizzino, A. Tullini, G. Gobbi, M. Santucci

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The case of a 7-year-old girl, born from a monochorionic biamniotic pregnancy (with healthy male twin) is presented. The patient showed a congenital brown-yellowish, raised, rough and oval-shaped nevic neoformation of the right temporo-zygomatic region. At 28 days of age she had a right hemiclonic status epilepticus, and from the 8th month of life she presented right sided partial motor seizures. Moreover from the beginning, her development milestones were delayed. When she was 5 years old an electroencephalogram displayed a sharp asymmetrical background activity, a continuous paroxysmal activity on the right hemisphere and independent focal irritative anomalies on the left posterior region. Her computed tomographic scan demonstrated megalencephaly on the right hemisphere. A biopsy specimen of her skin lesion showed the histological characteristics of a nevus sebaceous of Jadassohn. Taken together the clinical, neuroradiological and dermatological data led to the diagnosis of linear nevus sevaceous syndrome.

Original languageEnglish
Pages (from-to)55-58
Number of pages4
JournalGiornale Italiano di Dermatologia e Venereologia
Issue number1-2
Publication statusPublished - 1989

ASJC Scopus subject areas

  • Dermatology


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