Facial plexiform neurofibroma in a 13-year-old girl with neurofibromatosis-1

Abstract

Neurofbromatosis (NF) is an autosomal dominant disorder that affects the bone, the nervous system, soft tissue and the skin. NF is a neurocutaneous condition that can involve almost any organ system existing in two subtypes. NF1 is the most common subtype and is referred to as peripheral NF. Plexiform neurofibromas are diffuse, elongated fibromas coursing along the nerves. These lesions frequently involve the trigeminal or upper cervical nerves- Though plexiform neurofibroma occurs in only 5% of patients with NF 1, it is considered pathognomonic feature of NF1. We present a case of plexiform neurofibroma in the left side of the face in a 13-year-old girl with NF1.

Keywords

• Type I neurofibromatosis
• Plexifonn neurofibroma
• von Recklinghausen′s disease
• Café au lait macules