Bilateral segmental neurofibromatosis with speckled lentiginous nevus

A 41-year-old Korean man presented with multiple non-tender nodules on the anterior chest and back that had been present for 20 years. Physical examination revealed multiple, flesh-colored soft nodules varying in diameter from 0.4 to 2.5 cm, and confined to the T6 and T7 dermatomal area bilaterally (Figs. 1A, B). In addition, a light brown patch of 1.8 cm in diameter, speckled with smaller, darker colored macules, was found in the same dermatome (Fig. 2). No cafe-au-lait spots, intertriginous freckles, or Lisch nodules were detected. Family history (parents and two children) did not disclose any neurofibromas or abnormal pigmentation. A biopsy specimen of one of the nodules showed a circumscribed, nonencapsulated neurofibroma (Fig. 3). The speckled macule revealed hyperpigmentation in the basal layer and an ill-defined spindle-cell tumor in the deep dermis (Fig. 4).