Le syndrome de Schimmelpenning est un syndrome du naevus épidermique
Le syndrome du naevus épidermique, ou syndrome de Solomon, est composé de plusieurs entités dont le point commun génétique est le mosaïcisme.
Il comprend :
- le syndrome proteus,
- le CHILD syndrome (congenital hemidysplasia with ictyosiform erythroderma and limb defect),
- le syndrome du naevus comédonien,
- le syndrome du naevus de Becker
- le syndrome de Schimmelpenning.
- angora hair nevus syndrome
- Type 2 segmental Cowden disease
- FGFR3 epidermal nevus syndrome
- la phacomatose pigmento‐kératosique
syndrome de Schimmelpenning
The disorder consists of a triad of linear sebaceous nevus, seizures and mental retardation
syndrome de Schimmelpenning et IRM
 Neuroimaging findings in SFM syndrome include the primary lesions: Malformations of cortical development like hemimegalencephaly, polymicrogyria; gliomatosis, hemiatrophy with or without parenchymal cysts, vascular malformations and intracranial/intraspinal lipomas and secondary features: Porencephaly, infarcts and atrophy.  Other rare findings include enlarged ventricles, cortical hypoplasia, intracerebral calcification, arachnoid cysts, intracranial tumors and skull asymmetry.  CNS complications are more likely to be associated with epidermal nevi on the head and the CNS abnormalities are most often ipsilateral to the skin lesions.  Though hemiatrophy has been reported before in SFM, parenchymal cysts in brain have rarely been reported in SFM syndrome. There is mention of porencephalic cysts in the literature.  but no report of parenchymal cysts like the ones seen in our case.
Schimmelpenning Syndrome: A Kind of Craniofacial
Epidermal Nevus Associated with Cerebral and Ocular
MR Imaging Abnormalities