Are there pigmentation disorders associated with neurofibromatosis?

Yes. Common pigmented lesions of neurofibromatosis 1 (NF1, von Recklinghausen’s disease) are café-au-lait macules (CALM) and inguinal and axillary freckling. CALM are, as the name would imply, flat, tan to light brown lesions located anywhere on the body; these range from a few millimeters to several centimeters in diameter. Greater than 90% of CALM of NF1 are present at birth or appear within the first year of life. CALM are relatively common, and a patient must have six or more for the diagnosis of NF1 to be considered. If six or more are present, the absolute number of CALM does not correlate with the development of other systemic problems associated with NF1. NF1 is a relatively common autosomal dominant disorder that occurs in 1:3000 live births.