This disease is a spectrum of three sub-types including eosinophilic granuloma, hand-schuller-christian disease
and letterer-siwe disease.
DISCUSSION: Histiocytosis X is a clonal proliferation disease of langerhan's cells that express an immunophenotype positive for S100 and CD1a (7) Hand-schuller-christian disease
is a well described entity which has appeared sporadically till 1935.
is the chronic systemic variant, and Letterer-Siwe disease is an acute, fulminant, systemic condition.
Both terms lump together various diseases previously called Hand-Schuller-Christian disease
, Letterer-Siwe disease, and eosinophilic granuloma of the bone.
1] In 1868, Paul Langerhans discovered epidermal dendritic cells and the conditions which were characterized by these cells were subsequently called different names like Hand-Schuller-Christian disease
, Letterer-Siwe disease, and eosinophilic granuloma.