SCHIMMELPENNING SYNDROME: A CASE REPORT

A 6-year-old girl with a history of epilepsy was referred due to a mandibular lesion. Physical examination revealed the presence of sebaceous nevi involving skin of the face and neck. Intraoral exam revealed an asymptomatic swelling, firm to palpation, covered by mucosa of normal color, in the anterior mandible. The cone beam tomography revealed a presence of 3 well-defined mixed images, associated with impacted teeth 81, 82, and 42. The lesions were surgically excised and the histologic diagnosis was of adenomatoid odontogenic tumors (AOTs). Considering the multiple intraosseous neoplasms and the neurologic and epidermal affections, the diagnosis of Schimmelpenning syndrome was established. After 4 years, a lesion was found in the anterior region of the maxilla with clinical and imaging aspects similar to those of the previous lesions. After enucleation, the histologic analysis again indicated AOT. In 1 year of follow-up evaluation, no signs of recurrence were found. Support: FAPEMIG.