Segmental Neurofibromatosis: Is It Uncommon or Underdiagnosed?

Segmental neurofibromatosis (SNF) was described by Crowe et al1in 1956 and termedsectorial neurofibromatosis. Some years later, Miller and Sparkes2changed this term to SNF. The clinical peculiarities of SNF were established by Riccardi3in his first classification: cafe au lait (CAL) spots and/or neurofibromas in a single unilateral segment of the body, with no crossing of the median line, no familial history, and no systemic involvement. Riccardi included SNF in his neurofibromatosis (NF) type V. Over the years, the number of published cases of SNF have increased, but not all of the cases published were strictly in conformity with Riccardi's classification. Thus, Roth et al4subdivided the SNFs into four subtypes: true segmental, localized with deep involvement, hereditary, and bilateral. Report of a Case. A 17-year-old man presented for the check up provided for enlisted men in the Italian Navy. From early childhood, he