Langerhans cell histiocytosis: update for the pediatrician

Langerhans cell histiocytosis is the commonest of the histiocytic disorders. Owing to the relative rarity of the condition, it remains a disease in which the diagnosis is often delayed or missed and in which many questions remain unanswered, ranging from etiology and pathogenesis to therapy. The management is often frustrating for care-givers and parents/patients. The purpose of the review is therefore to raise awareness of the disease and to highlight the clinical findings that should make the pediatrician or primary care-giver suspect the diagnosis, as well as current thinking regarding management of the various and diverse manifestations of this disease.

We discuss new and interesting insights into the biology of Langerhans cell histiocytosis that raise the possibility of future targeted therapy. Important points in the diagnosis, investigation and management of the various forms of the disease are also discussed.

We present a review of childhood Langerhans cell histiocytosis, highlighting new insights into pathogenesis and management of the various forms of this complex disease.