Therapy is often challenging. Epidermal nevi are usually resistant to topical and intralesional steroids, dithranol, topical retinoids, and cryosurgery. Topical calcipotriol may be effective. In the United States, calcipotriol is not approved for children younger than 12 years. [28, 29]
For Schimmelpenning syndrome, antiepileptic medications (if needed) should be promptly considered.
Inpatient care is indicated only when potentially serious or life-threatening medical problems result from internal defects of the brain, eyes, or skeleton.
If the size and the site are suitable, the nevus may be excised if desired by the patient. 
A neonate with seizures, linear sebaceous nevus syndrome, and hemimegalencephaly benefited from a hemispherectomy at 36 weeks' gestational age. 
Carbon dioxide laser therapy for linear nevus sebaceous in the Schimmelpenning-Feuerstein-Mims syndrome may be beneficial. 
For nevus comedonicus syndrome, if an associated cataract is present, consult an ophthalmologist for therapy.
A newborn girl was documented with a right-sided extended epidermal nevus, congenital rhabdomyosarcoma of the inguinal area, and hemihypertrophy. One should watch for a spectrum of organ system involvement in epidermal nevus syndrome at a very early age life. 
Facial paralysis due to an inflammatory pseudotumor of the facial nerve as a rare complication of epidermal nevus syndrome has been described. 
Epidermal nevus syndrome is a sporadic neurocutaneous linkage of congenital ectodermal defects and cannot be prevented.
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