in 1895 jadassohn was the first to describe congenital cutaneous lesions in a linear distribution , for which he coined the term of naevi sebacei. epidermal nevus syndrome ( ens ) has been used to describe the association of an epidermal nevus with systemic features ( potentially affecting multiple organs including neurologic , ocular , skeletal , and in rare cases , cardiac and renal anomalies ) . we report a case of epidermal nevus syndrome with extensive skin lesions , cns and eye anomalies in a full term infant who was conceived by intracytoplasmic sperm injection ( icsi ) . full - term male neonate , conceived by icsi , was born to a 21-year - old mother ( gravida 1 , para 1 ) at 39 weeks of gestation by caesarean delivery and resuscitation was assigned as uneventful . a prenatal sonogram at 32 weeks gestation revealed polyhydraminos as well as a well - defined hyperechoic mass 4.53 cm in the left lateral side of the face and neck with central cystic area . the initial physical examination showed normal growth parameters with an evident cutaneous abnormality of the face consisted of linear , well - demarcated , raised , yellowish , fleshy and hairless plaques with velvety surface located on the left side of the forehead , the parietal region of the scalp , the face , chin , lower lip , and left ear with a smaller lesion on right side of face [ figure 1 ] . a similar , less well - formed lesion was present over the midline of the back at the lumbar and the sacral regions and scrotum . also , a black nodule was noted over the medial canthus of the right eye . there were no other cutaneous manifestations , such as cafe - au - lait spots or hypo- or hyper - pigmentation lesions . well - demarcated , raised , velvety plaques on left side of face and ear with other linear ones in medline of face and chin magnetic resonance imaging ( mri ) of his brain revealed an extra - axial csf signal intensity lesion in the left side of the posterior fossa and the cistern magna suggesting arachnoid cyst with mild left cerebellar hypoplasia ( dandy walker variant ) [ figure 2 ] . abdominal sonogram , doppler of renal vessels , bone survey and echocardiography revealed normal results . also , serum calcium , phosphorus and urinary calcium were normal . mri brain t1-weighted with axial plane showed extra - axial csf signal intensity lesion in the left side of the posterior fossa and the cistern magna suggesting arachnoid cyst with mild left cerebellar hypoplasia ( dandy walker variant ) histopathology showed thick hyperplastic epidermis with underlying large immature sebaceous lobules and free cut margins with no evidence of cellular atypia . during hospitalization of the patient , no seizures occurred and no medication was prescribed . was obtained from the parents of our patient for publication of this case report and any accompanying images . a copy of the written consent signed by the father of our patient is available for review by the editor - in - chief of this journal . linear nevus sebaceous syndrome ( lnss ) includes sebaceous nevus and defects of the brain , eyes , or bones . estimated incidence of epidermal nevi is about 1 - 3 per 1000 live births , with no sexual predilection . nevus sebaceous , which is a hallmark of lnss , is a hamartoma of the epidermis , hair follicles and sebaceous and apocrine glands . it is usually presents at birth as a yellow - orange to pink , finely papillomatous , alopecic plaque that is often oval or linear . the lesions vary in size from a few millimeters to several centimeters in length , but unusually large and exophytic lesions had been reported . there is a predilection for the scalp and the face , but they may also occur on the neck and trunk . during puberty , they have a tendency to become more raised , verrucous , and greasy , at least in part because of androgen stimulation of sebaceous glands . the most frequent extra - cutaneous manifestation of nevus sebaceous syndrome is cns involvement , including seizures , developmental delay , and structural brain abnormalities . the structural brain abnormalities may include hemimegalencephaly , cerebral heterotopias , agenesis of corpus callosum , and dysplasia of brain vessels or dandy walker malformation . indeed , it has been suggested that the syndrome could be considered an oculo - neuro - cutaneous syndrome . the ocular lesions include choristoma of conjunctiva , limbal dermoids , colobomas of the eyelid , iris , and retina , cataracts , strabismus , ptosis , microphthalmos , macrophthalmos , absence of the ganglion cell layer , nystagmus and optic nerve hypoplasia . our patient has extensive nevus sebaceous with a linear configuration that involved the left side of the forehead , scalp , face , back and genital area , left cerebellar hypoplasia ( dandy walker variant ) , and bilateral posterior polar cataract . these findings are consistent with the diagnosis of lnss and further support the possibility of this syndrome to be part of oculo - neuro - cutaneous syndromes . a range of musculoskeletal , cardiovascular , and urogenital manifestations have been described with lnss . nearly all cases are sporadic , but occasionally , nevus sebaceus may affect several members of a family . when post fertilization loss of heterozygosity occurs in a somatic cell , a homozygous or hemizygous cell clone would originate and give rise to a mosaic patch . it has been suggested that the earlier the mutation event occurs in embryogenesis , the more deleterious consequences will be manifesting as nevus sebaceous syndrome . also , hpv infection of a pluripotent stem cell at an early stage of embryogenesis could play a role in the pathogenesis of nevus sebaceous syndrome . hansen and colleagues found that infants conceived with use of intracytoplasmic sperm injection or in vitro fertilization have twice as high a risk of a major birth defect as naturally conceived infants . the icsi procedure itself or using sperm from sub - fertile men may increase the potential to transmit genetic or chromosomal , either sex or autosomal , aberrations to offspring created from icsi technology . reported malformations with icsi although skin nevi were previously reported with icsi , to the best of our knowledge this is the first case to describe the association between lnss and icsi . although , surgical excision is the treatment of choice , a multidisciplinary approach is mandatory . the pediatrician , neurologist , ophthalmologist , dermatologist , and geneticist should give advice .
linear nevus sebaceous syndrome is a multisystem disorder including nevus sebaceous , which is the hallmark of this syndrome , together with central nervous system , ocular and skeletal anomalies . 
 we report a case of extensive skin lesions , cns and eye anomalies in a full term infant who was conceived by intracytoplasmic sperm injection .