Abstract
Segmental neurofibromatosis (SNF) is a rare manifestation of neurofibromatosis. Very few cases have been reported in older population. Here, we present a case of 73 year old male with four years history of multiple, skin coloured, dome shaped, soft nodules situated over left lumbar region. The histopathological evaluation of the excised nodule revealed neurofibroma. Recognition of this condition is important, as there may be associated systemic involvement, malignancies and possibility of transmission to the offspring.
Highlights
Segmental neurofibromatosis (SNF) is a rare manifestation of neurofibromatosis characterized by cutaneous lesions usually limited to one body region, and an absence of systemic complication in the majority of patients.[1]
In SNF, there is a risk of developing neurofibromatosis type 1 (NF1) in the offspring with systemic involvement like oculocutaneous lesions, bony abnormalities, seizure, and learning difficulties.[1,4]
Riccardi established the diagnostic criteria for SNF: pigmentary abnormalities, which includes café-au lait macules and/or axillary freckles, and/ or neurofibromas in a single unilateral segment of the body, with no crossing of midline, no family history of neurofibromatosis and no systemic involvement.[5]
Summary
Segmental neurofibromatosis (SNF) is a rare manifestation of neurofibromatosis characterized by cutaneous lesions usually limited to one body region, and an absence of systemic complication in the majority of patients.[1]. In SNF, there is a risk of developing neurofibromatosis type 1 (NF1) in the offspring with systemic involvement like oculocutaneous lesions, bony abnormalities, seizure, and learning difficulties.[1,4]
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