Histiocytosis, Langerhans Cell
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C96.5 – Multifocal and unisystemic Langerhans-cell histiocytosis
C96.6 – Unifocal Langerhans-cell histiocytosis
277.89 – Other specified disorders of metabolism
Langerhans cell histiocytosis (LCH) is a group of rare idiopathic disorders in which dendritic histiocytes accumulate in one or many organs. Patients have previously been classified as having histiocytosis X, Hand-Schüller-Christian syndrome, Letterer-Siwe disease, Hashimoto-Pritzker disease, self-healing reticulohistiocytosis, or eosinophilic granuloma; however, patients are now classified based on the number of organ systems involved (ie, single-system or multisystem LCH). Organs may include the skin, oral mucosa, bone, bone marrow, lungs, liver, spleen, gastrointestinal tract, lymph nodes, or central nervous system. LCH most commonly affects children aged 1-4 years but may present from birth to adulthood. The 3-year survival rate is approximately 80% with age under 2 years, multiorgan involvement, and organ dysfunction portraying a worse prognosis.