Dermatological features
The dermatological manifestations of type I neurofibromatosis include:
- cafe-au-lait spots:
- six or more of 0.5 cm in diameter is characteristic
- usually develop during the first year of life but may be present from birth
- Crowe's sign:
- bilateral axillary and inguinal freckling
- bilateral axillary and inguinal freckling
- cutaneous neurofibromas:
- develop in late childhood
- soft, sometimes pedunculated
- appear to be "button holed" through the skin
- may progress to become plexiform neurofibroma, mollusca fibrosa or elephantiasis neurofibromatosa.
- Some lesions may become very large.
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