Abstract
Atypical presentation of Schimmelpenning-Feuerstein-Mims syndrome associated to a giant congenital nevus: a one case report
Highlights
Schimmelpenning-Feuerstein-Mims (SFM) syndrome is a rare congenital neurocutaneous disorder characterized by the association of sebaceous hamartoma with extracutaneous abnormalities in different neuroectodermal organ systems.Its association with a giant congenital nevus has never been reported so far
We report the first case of an SFM syndrome associated with a giant congenital nevus in the neonatal intensive care unit (NICU) of Mohamed VI University Hospital of Marrakesh
The dermatological examination revealed a multiple yellow-orange lesions with a warty surfaces of geographical shapes on the scalp, the face and the neck evoking the sebaceous hematoma, in addition to a giant melanocytic congenital nevus which was extended to the entire body sparing the hands and feet, surmounted by medium-abundance hairiness associated with contact bleeding nodules, sebaceous scalp and facial hamartomas, and a large pilot liparus nevus (Figs. 1 and 2)
Summary
Schimmelpenning-Feuerstein-Mims (SFM) syndrome is a rare congenital neurocutaneous disorder characterized by the association of sebaceous hamartoma with extracutaneous abnormalities in different neuroectodermal organ systems. We report the first case of an SFM syndrome associated with a giant congenital nevus in the NICU of Mohamed VI University Hospital of Marrakesh. We report a case of a term male newborn, born by vaginal delivery, from a first degree consanguineous marriage, his mother aged 22-years, had 2 gestity, 2 parity, 2 children alive, the older brother aged one and a half years old with no reported abnormalities. Admitted in his first day of life to the NICU; the newborn suffered from respiratory distress and neonatal infection. The death occurred at his twenty-fifth day of hospitalization, caused by uncontrolled severe sepsis
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