Among the potential sites of involvement by Langerhans cell histiocyto
sis (LCH), the head and neck region is the most commonly cited. Though
principally a pediatric disease, LCH can affect any age group. It can
be unifocal (skeletal) or multifocal (skeletal and/or visceral); it a
ppears as though the presence of visceral lesions is more common in th
e youngest patients, and may be associated in some with a rapidly prog
ressive course resulting in death. Head and neck manifestations may mi
mic such varied entities as eczema, otitis media, osteomyelitis, and c
holesteatoma. Current approaches to therapy are less aggressive than t
hey were in the past, and are particularly intended to monitor for and
treat any complicating secondary infections (which may develop in the
youngest patients with multifocal disease including visceral involvem
ent). The prognosis is very good for unifocal skeletal system disease,
and poor for multifocal disease with involvement of tissues other tha
n bone.