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Genetic and Rare Diseases Information Center (GARD)

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Langerhans cell histiocytosis

Other Names for this Disease
  • Eosinophilic granuloma (formerly)
  • Hand-Schüller-Christian syndrome (formerly)
  • Histiocytosis X
  • LCH
  • Letterer-Siwe disease (formerly)
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What are the signs and symptoms of Langerhans cell histiocytosis?

Symptoms of Langerhans cell histiocytosis (LCH) can vary greatly from person to person depending on how much of the body is involved and what part(s) are affected. The disease can affect virtually every organ, including skin, bones, lymph nodes, bone marrow, liver, spleen, lungs, gastrointestinal tract, thymus, central nervous system, and hormone glands. The symptoms may range from localized bone lesions or skin disease to multiple organ involvement and severe dysfunction.[1]

Below are the organs that may be affected as well as the symptoms that might be observed:[1]

  • Skin - Red, scaly papules in areas where opposing skin surfaces touch or rub (e.g. skin folds) are commonly seen in LCH. Infants with the skin presentation on the scalp are often misdiagnosed with cradle cap. The skin symptoms usually improve without treatment.
  • Bone - Lesions that cause bone destruction are common, with the skull, lower limbs, ribs, pelvis, and vertebrae usually being affected. Symptoms may include pain, swelling, limited motion, and inability to bear weight.
  • Lymph node - Lymph node involvement may be limited or associated with a skin or bone lesion or disseminated disease. Although any of the lymph nodes may be affected, the cervical lymph nodes are where the disease commonly occurs. Individuals usually only present with pain of the lymph node affected. If only one lymph node is affected, prognosis is normally good and treatment is unnecessary.
  • Liver - Liver involvement at the time of diagnosis is generally associated with more severe disease. Symptoms may include ascites, jaundice, low levels of protein, and prolonged clotting time.
  • Central nervous system (CNS) and hormone - CNS involvement is rare and may be devastating. The most common result of CNS involvement is the altering of hormonal function, with some individuals developing diabetes insipidus.

More detailed information about the symptoms of LCH can be accessed through the Histiocytosis Association's website.

Last updated: 12/2/2013

  1. Grifo AH. Langerhans Cell Histiocytosis in Children. Association of Pediatric Hematology/Oncology Nurses. 2009;