Please click to enlarge.
Note what signs you see.
I (W. Wertelecki, M.D.) see three images of an extensive plexiform neurofibroma. Note that on the face there is no evidence of café-au-lait (CaL) spots nor of neurofibromas. However, there is a large invasive plexiform neurofibroma in the peri-auricular region. On palpation, this region is hyperthermic in comparison to contra-lateral area.
In the center is illustrated the ear area – it is edematous, there is a patch of melanic hyperpigmentation and areas of neo-tricho-genesis (plainly stated, new hair growth – please enlarge the image).
Partial surgical resection of the tumor was feasible in the scalp area.
PERSPECTIVE: In contrast to the trunk where CaL and neurofibromas are most frequent, the face is often virtually not or minimally impacted. This is not so regarding plexiform neurofibromas. Please note, we have postulated a hypothesis of a potential relation with temperature (see Clinical Notes and Points). Please also see similar patients in the NF1 gallery of signs and a recent posting.