The Elephant Man : Human Monstrosity Or Marvel?

Sihlpa Deoke, Ramesh Mundle, Sony Thomas

Abstract


Neurofibromatosis type 1 is an inherited neuro-cutaneous syndrome. Described by Friedrich von Recklinghausen for the first time in 1882, it is characterized by benign tumors (neurofibromas) arising from neural sheath cells, subcutaneous, visceral peripheral or cranial nerves and cutaneous manifestations like cafe-au-lait spots. F'lexiform Neurofibromatosis (PNF) is seen exclusively in neurofibromatosis type 1. PNF is common along trigeminal nerve though segmental plexiform neurofibromas in scalp, neck, chest, pelvis of abdomen have been reported. When present along the Trigeminal never, it can cause gross disfigurement a cosmetic as well as functional problems. We hereby report a case of facial plexiform neurofibromatosis.
Key Words: - Neurofibromatosis type 1, plexiform neurofibromatosis, café-au-lit spots
 

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