Well circumscribed, raised yellow/brown firm papule or nodule, typically solitary. Can be congenital but otherwise typically very young boys, head and neck area, asymptomatic.

Can affect the iris – presents with a red eye…

Can ulcerate, otherwise they tend to atrophy and disappear after 3-6 years.

Seen in 10% of Neurofibromatosis type 1.

Can rarely be multiple and internal (liver, bone marrow etc). Screening of asymptomatic cases probably only justified if multiple.

Differential – mastocytoma, Langerhans histiocytosis, molluscum.