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Segmental neurofibromatosis in an octogenarian
1Department of Medicine, UMDNJ/Robert Wood Johnson Medical School, Camden.
Journal of the American Geriatrics Society
|July 1, 1990
Summary
Segmental neurofibromatosis (SNF), a rare variant, is typically diagnosed in younger individuals. This report details the first known case presenting in an octogenarian, highlighting its late onset and unique characteristics.
Area of Science:
- Dermatology
- Medical Genetics
Background:
- Neurofibromatosis is a group of genetic disorders characterized by the development of tumors in the nervous system.
- Segmental neurofibromatosis (SNF), classified as NF-V by Riccardi, is a rare, non-hereditary variant typically manifesting in childhood or early adulthood.
Observation:
- This case report describes an octogenarian patient who first noticed neurofibromatosis lesions at age 74.
- The patient presented with features consistent with segmental neurofibromatosis.
Findings:
- The patient's late-onset segmental neurofibromatosis represents the earliest documented presentation of this variant in the eighth decade of life.
- This case underscores that SNF can manifest significantly later in life than typically observed.
Implications:
- Geriatric physicians should consider SNF in the differential diagnosis of cutaneous lesions in elderly patients.
- Awareness of SNF's late-onset potential is crucial for accurate diagnosis and management in the geriatric population.
- The lack of genetic heritability and systemic manifestations in this case is important for patient counseling and prognosis.