Establishing the diagnosis
- Epidemiology
- Results from proliferation of all elements of a peripheral nerve
- Increased incidence with age, most common over 40–50 (Font, AFIP)
- Rare familial forms exist
- Pertinent elements of the patient history
- Gradual onset
- Chronic course
- Clinical features
- Neural tumor that can affect skin in all parts of the body
- Solitary lesions are unassociated with systemic disease
- Multifocal or diffuse lesions are associated with type 1 neurofibromatosis (Shields 2008)
- Eyelid and orbit can be involved by
- Solitary neurofibroma (fibroma molluscum)
- Multiple localized neurofibromas
- Plexiform neurofibroma (Shields 2008)
- Plexiform neurofibromas develop in young children as a thickening of the entire eyelid that causes an S-shaped curve to the margin of the upper eyelid (Figure 4)

Figure 4. Eyelid neurofibroma.
- Plexiform neurofibromas can extend into the eyebrow, conjunctiva, and orbit
- Testing and evaluation to establish diagnosis
- Diagnosis established by histopathology
- Composed of combination of Schwann cells, peripheral nerve axons, neural fibroblasts, and perineural cells (Bechtold 2012)
Risk factors
Type 1 neurofibromatosis (von Recklinghausen's disease)
Differential diagnosis
- Chalazion
- Subcutaneous or orbital capillary hemangioma
Clinical management
- Observation of small, asymptomatic solitary neurofibromas
- Surgical excision or debulking
- Use of CO2 laser at time of surgical resection for hemostasis (Bechtold 2012)