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Segmental neurofibromatosis.
Chris G Adigun1, Jennifer Stein
1Department of Dermatology, New York University, New York, New York, USA.
Dermatology Online Journal
|October 28, 2011
Summary
Segmental neurofibromatosis (SNF) is a rare condition characterized by neurofibromas in a dermatomal pattern. This case highlights SNF diagnosis in a patient without other neurofibromatosis symptoms, requiring no medical treatment.
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) is a rare, typically sporadic form of neurofibromatosis, often presenting with localized neurofibromas.
Observation:
- A 59-year-old man presented with non-tender, fleshy nodules in a dermatomal distribution on his left chest and axilla.
- Biopsy confirmed the nodules as neurofibromas.
Findings:
- The patient met the diagnostic criteria for segmental neurofibromatosis (SNF) based on Riccardi's classification.
- The diagnosis was supported by the dermatomal arrangement of neurofibromas, absence of other cutaneous findings, and no family history of neurofibromatosis.
Implications:
- This case underscores the importance of recognizing SNF based on clinical presentation and distribution patterns.
- As the patient exhibited no complications of neurofibromatosis type 1, no specific medical treatment was indicated, emphasizing a conservative management approach for uncomplicated SNF.
