Two cases of segmental neurofibromatosis

초록

Neurofibromatosis is now regard as a heterogenous disease. Crowe et al, assigned the term sectorial to broad category of neurofibromatosis with limit distribution. Some years later, Miller and Sparkes changed this term to segmental neurofibromatosis(SNF). The clinical peculiarities of SNF were established by Riccardi in his first classification: cafe au lait 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 type V. A 34-year-old man had several nodule on right T5 and T6 dermatome which was presented at 25 year of age. A woman, age 53, had unilateral multiple nodule on right T8 and T9 dermatome since 20 years before. There were not cafe-au-lait spots, axillary frecklings, or Lisch nodule in both cases. Biopsy specimen showed collagenous wavy fibrils among which spindle-shaped and round cells were embedded as seen in the typical neurofibroma. We reported two cases of typical segmental neurofibromatosis, with reviewed the literatures.