Lineal nevus sebaceous syndrome regarding two cases
Keywords:
linear sebaceous nevus, nevus, sebaceous of Jadassohn, Schimmelpenning syndromeAbstract
Introduction: Linear nevus sebaceous syndrome is a rare neurocutaneous disease that results from genetic mosaicism with a dominant gene. It is characterized by a wide spectrum of anomalies with multisystem involvement; alterations of the central nervous system are the most frequent extracutaneous manifestations, with intellectual disability and epileptic seizures being the most common.
Objective: To describe the clinical evolution, the results of complementary examinations and the response to anti-seizure treatment in two patients with linear sebaceous nevus syndrome.
Case presentation: Case 1 was a 13-year-old male patient with linear Sebaceous Nevus on the face (midline and left hemiface), area of midline alopecia, left microphthalmia and corneal opacity, and spastic quadriparesis. Debuted at three months old with West syndrome that evolved into Lennox-Gastaut Syndrome. Case 2 was a 19-year-old female patient with linear sebaceous nevus on the face (midline), craniosynostosis, dysmorphisms, focal epileptic seizures, and intellectual disability.
Conclusions: It is a rare, underdiagnosed entity, with multisystem involvement; cause of drug-resistant epilepsy and intellectual disability. Its diagnosis and treatment must be individualized and requires interdisciplinary management.
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Copyright (c) 2025 Aisel Santos Santos, Leidy García Morales, Cristin María del Mar Arias Cortes, Dilenny Rocio Peguero Velasquez, Juan Carlos Padilla Reyes, Karenia Joglar Hernandez
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