The mystery of brown shawl over back: a rare case of giant plexiform neurofibroma underlying a giant café-au-lait macule

Abstract

Neurofibromatosis is a rare autosomal dominant disorder characterized by café-au-lait macules and cutaneous neurofibromas. Café-au-lait spots are sharply defined, light-brown colored homogenous macules observed in more than 90% of cases. Plexiform neurofibromas are benign tumors originating from nerve sheath cells, subcutaneous or visceral peripheral nerves involving multiple fascicles often described on palpation as a bag of worms. We describe a case of 25-year-old male who presented with a giant plexiform neurofibroma underlying a giant café-au-lait macule.

Keywords

• bag of worms
• café-au-lait macule
• malignant peripheral nerve sheath tumor
• neurofibromatosis
• plexiform neurofibroma