Bilateral Segmental Neurofibromatosis with Partial Unilateral Lentiginosis: Case Report with Review of the Literature

Bilateral segmental neurofibromatosis is a rare disease characterized by bilateral neurofibromas, with or without pigmented lesion, or unilateral neurofibromas with contralateral pigmented lesion, limited to a body segment. Partial unilateral lentiginosis is characterized by numerous lentigines localized to a body segment, often corresponding to one or more dermatome. Bilateral segmental neurofibromatosis combined with partial unilateral lentiginosis is very rare, and to our knowledge, only 2 cases have been reported in English literature. We herein report another case of bilateral segmental neurofibromatosis with partial unilateral lentiginosis in a 46-year-old woman.

Bilateral Segmental Neurofibromatosis Showing Different Dermatomal Distribution

A 43-year-old woman presented with numerous cutaneous neurofibromas, limited to the left anterior chest(T2-3) and the right lower back(L1-2). These had been present for 10 years. Neither cafe-au-lait spot, intertriginous freckle, nor Lisch nodule was found. The family history was negative for neurofibromatosis. Biopsy specimens showed circumscribed, nonencapsulated neurofibromas. The present case was a rare form of bilateral segmental neurofibromatosis in that while most of the reported cases involved the same dermatome bilaterally, she had bilaterally different dermatomal neurofibromas.

A Case of Bilateral Segmental Neurofibromatosis Associated with Partial Unilateral Lentiginosis and Nevus of Ota / 대한피부과학회지

The term, bilateral segmental neurofibromatosis had been used to refer to patients who had unilateral neurofibromas with contralateral pigmented lesions or bilateral neurofibromas. Partial unilateral lentiginosis is characterized by numerous lentigines localized to a body segment. The coexistence of bilateral neurofibromas and partial unilateral lentiginosis raises the possibility that partial unilateral lentiginosis could be a variant of segmental neurofibromatosis. Nevus of Ota arise from dermal melanocytes and it can be associated with neurofibromatosis. A 60-year-old man presented with bilateral segmental neurofibromatosis with partial unilateral lentiginosis on the right arm and right leg, nevus of Ota on right forehead, and one cafe-au-lait spot on the abdomen.