Segmental neurofibromatosis with only macular lesions

A 16-year-old girl had a three-year history of many café au lait spots and freckles in a dermatomal distribution. The diagnosis of segmental neurofibromatosis (NF-5) was made on the basis of the clinical features, distribution of the lesions, and absence of systemic involvement. Only a few cases in the literature describe NF-5 with only macular lesions. An accurate clinical examination is important to identify this unusual presentation.