Histiocytosis X or Langerhans cell histiocytosis (LCH) is a disease that po
ssesses three less distinctive and overlapping states called eosinophilic g
ranuloma (EG)I Hand-Schuller-Christian (HSC) disease and Letterer-Siwe (LS)
disease. EG is the least severe and localized form of all LCHs and possess
es the best prognostic result. A high index of suspicion is required to dia
gnose the EG, especially when an ear disease is refractory to medical treat
ment. Early detection is important to manage the EG properly and to minimiz
e the complications or sequels of treatment. Definitive diagnosis of histio
cytosis is made by histopathological means and immunohistochemical detectio
n of S-100 and CD1 antigens in the tissue samples. And differential diagnos
is of the subgroups is made according to the clinical manifestations such a
s visceral organ or bone involvement. Surgical excision, radiotherapy and c
hemotherapy, either alone or in combination, are the main treatment options
. (C) 2001 Elsevier Science Ireland Ltd. All rights reserved.