History and Nomenclature of LCH

Many eponyms and classifications have been used to describe the various forms of LCH in different eras – eosinophilic granuloma, (Abt-)Letterer-Siwe, Hashimoto-Pritzker and Hand-Schu(e)ller-Christian Disease.

  • In 1953, Lichtenstein coined the term Histiocytosis-X for the different forms of Langerhans cell histiocytosis — the X denoting his uncertainty about the disease and it’s pathogenesis.
  • In 1964, Mr F Basset demonstrated that the histiocyte responsible of this entity is a cell with Langerhans cell features – a macrophage homing naturally in the skin.
  • Since the seventies, with the contribution of F Basset and C Nezelof, the term Histiocytosis X has been progressively replaced by the denomination Langerhans cell histiocytosis.

In 1989, the Histiocyte society proposed a summary of the diagnosis criteria. While the presentation of LCH is very variable, the histopathology is the same in all diseases and syndromes summarized under LCH: There is an accumulation of histiocytes (= macrophages) which present specific markers of the Langerhans cells (CD1a, Langerine and Birbeck granuloma).