Oral Sirolimus Treatment for HRAS-Related Epidermal Nevus Syndrome.

This is the case of a 10-year-old female with HRAS-related epidermal nevus syndrome with extensive and severe cutaneous involvement treated with oral sirolimus. The patient had progressively enlarging verrucous periorbital plaques involving the eyelids, resulting in lagophthalmos and threatening vision. Oral sirolimus rapidly improved the cutaneous lesions and resolved the lagophthalmos. This case of a patient treated with oral sirolimus for HRAS-related epidermal nevus syndrome suggests that therapies targeting the mTOR pathway may indirectly affect the RAS/MAPK pathway.