CENTRAL GIANT CELL LESION IN A PATIENT WITH HYPOPPHOSPHATEMIC OSTEOMALACIA ASSOCIATED WITH EPIDERMIC NEVUS SYNDROME

Abstract

Epidermal nevus syndrome (ENS) is a rare congenital condition characterized by epidermal nevi associated with skeletal, neurological, cardiovascular, ocular, and genitourinary abnormalities. Epidermal nevi have been considered producers of phosphaturic factors capable of triggering hypophosphatemic osteomalacia. The association between the systemic factors of ENS and how they influence the behavior of central giant cell lesions (CGCL) in the gnathic bones is not yet established. A 19-year-old female patient with hypophosphatemic osteomalacia as a result of ENS (epidermal nevi in the hemiface, trunk, and right limbs) presented with facial asymmetry caused by increased volume due to aggressive and extensive mandibular CGCL. Due to the impossibility of mandibular reconstruction with a microsurgical flap and the patient's endocrine condition, which contraindicated drug treatment with glucocorticoids or calcitonin, conservative osteoplasty was chosen to avoid the sequelae of a hemimandibulectomy. At postoperative follow-up (less than 3 years), the lesion was stabilized and there was bone neoformation.