FACIAL PLEXIFORM NEUROFIBROMATOSIS IN A 30 YEARS OLD PATIENT WITH NEUROFIBROMATOSIS-1: A CASE REPORT

Abstract

Background: Plexiform neurofibromatosis (PN) is a benign tumor that develops from peripheral nerve sheath, which can be found in 30% case of neurofibromatosis type 1 (NF1). PN which especially occurs in the face could cause disfigurement and functional problems. The main therapy for PN is surgery.

Case: A 30-year-old man came to the Department of Dermatovenerology of dr. Moewardi Hospital with a complaint of nodules in the face since 8 years ago which started as a little papule like a pimple which evolved into nodules that covers the left side of the face and the right chin and cheek. Other than these nodules, the patient also has brown spots since his birth that are spread all over his body. After a careful anamnesis, physical examination and a histopathological examination, the patient was diagnosed with plexiform neurofibromatosis in neurofibromatosis type 1. Therapy for this patient at the moment is ketotifen hydrogen fumarate 1 mg tablet twice daily and is planned for a serial surgical resection of the nodules.

Discussion: Plexiform neurofibromatosis is a benign nerve sheath tumor that is included as diagnostic criteria for NF1. The most common location is in the face and occurring hemifacial hence causing disfigurement and cosmetic problems. The main therapy is surgical resection but because the tumor is often found along the nerve and the infiltrative nature of the tumor, surgery is often difficult and there is a chance of recurrence in the future. Other than surgery, we can give symptomatic medication with an antihistamine such as ketotifen fumarate to reduce the pruritus.

KEYWORDS: Facial plexiform neurofibromatosis, Neurofibromatosis type 1, Ketotifen hydrogen fumarate.