Skip to content

    Children's Health

    Font Size
    A
    A
    A

    Langerhans Cell Histiocytosis

    Important
    It is possible that the main title of the report Langerhans Cell Histiocytosis is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

    Synonyms

    • LCH
    • histiocytosis X
    • Abt-Letterer-Siwe disease
    • eosinophilic granuloma
    • Hand-Schueller-Christian syndrome
    • Hashimoto-Pritzker syndrome
    • Letterer-Siwe disease
    • pure cutaneous histiocytosis
    • self-healing histiocytosis

    Disorder Subdivisions

    • None

    General Discussion

    Langerhans cell histiocytosis (LCH) is a spectrum of rare disorders characterized by overproduction (proliferation) and accumulation of a specific type of white blood cell (histiocyte) in the various tissues and organs of the body (lesions). The lesions may include certain distinctive Langerhans cells involved in certain immune responses, as well as other white blood cells (e.g.,lymphocytes, monocytes, eosinophils). Associated symptoms and findings may vary from case to case, depending upon the specific tissues and organs affected and the extent of involvement. Most often the bone lesions are painful. Skin rashes may itch or cause painful ulcers especially under the arms or groin area. The pathogenesis (medical cause) is not clearly understood and an ongoing debate continues regarding its cause as a reactive immunologic or neoplastic (cancer-like) process. No infectious agent (virus, bacteria, or fungus) has been associated with LCH. Patients often have a strong family history of immune diseases such as thyroid disease, arthritis, or lupus.

    Most affected individuals have single or multiple bone lesions characterized by lytic lesions (holes in the bones). Although the skull is most commonly affected, there may also be involvement of other bones, such as those of the spine (vertebrae) and the long bones of the arms and legs. Affected individuals may have no apparent symptoms (asymptomatic), or may experience associated pain and swelling, and/or develop certain complications, such as fractures or secondary compression of the spinal cord. Other organs may also be affected, including the skin, lungs, liver, spleen, bone marrow, thymus, thyroid,intestines and brain. In some individuals, LCH may be associated with involvement of the pituitary gland leading to diabetes insipidus, growth failure, hypothyroidism, or insufficitne production of sex hormones.

    Langerhans cell histiocytosis was selected by the Histiocyte Society to replace the older, less specific term histiocytosis X. Histiocytosis X encompassed three entities known as eosinophilic granuloma, Hand-Schuller-Christian disease, and Letterer-Siwe disease that were characterized by the accumulation of histiocytes. The "X" denoted that the cause and development of the disorder was not understood. Langerhans cell histiocytosis was chosen because it seemed that the Langerhans cells might play a central role in the development of these disorders. However, new research (Allen 2010) has shown that the skin Langerhans cell is not the cell of origin, but a myeloid dendritic cell.

    Resources

    Histiocytosis Association, Inc.
    332 North Broadway
    Pitman, NJ 08071
    Tel: (856)589-6606
    Fax: (856)589-6614
    Tel: (800)548-2758
    Email: association@histio.org
    Internet: http://www.histio.org

    March of Dimes Birth Defects Foundation
    1275 Mamaroneck Avenue
    White Plains, NY 10605
    Tel: (914)997-4488
    Fax: (914)997-4763
    Tel: (888)663-4637
    Email: Askus@marchofdimes.com
    Internet: http://www.marchofdimes.com

    American Lung Association
    1301 Pennsylvania Ave NW
    Suite 800
    Washington, DC 20004
    USA
    Tel: (202)785-3355
    Fax: (202)452-1805
    Tel: (800)586-4872
    Email: info@lungusa.org
    Internet: http://www.lungusa.org

    NIH/National Heart, Lung and Blood Institute
    P.O. Box 30105
    Bethesda, MD 20892-0105
    Tel: (301)592-8573
    Fax: (301)251-1223
    Email: nhlbiinfo@rover.nhlbi.nih.gov
    Internet: http://www.nhlbi.nih.gov/

    Genetic and Rare Diseases (GARD) Information Center
    PO Box 8126
    Gaithersburg, MD 20898-8126
    Tel: (301)251-4925
    Fax: (301)251-4911
    Tel: (888)205-2311
    TDD: (888)205-3223
    Internet: http://rarediseases.info.nih.gov/GARD/

    Histiocytosis Association of Canada
    Box 29095
    Okanagan Mission RPO
    Kelowan, BC, V1W 4A7
    Canada
    Tel: 2507646104
    Fax: 2507646104
    Email: histio.canada@shaw.ca
    Internet: http://www.histiocytosis.ca/

    Histiocytosis Research Trust
    PO Box 435
    Leeds, LS17 1GE
    United Kingdom
    Tel: 07850740241
    Email: lynn@hrtrust.org
    Internet: http://www.hrtrust.org

    For a Complete Report:

    This is an abstract of a report from the National Organization for Rare Disorders (NORD). A copy of the complete report can be downloaded free from the NORD website for registered users. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational therapies (if available), and references from medical literature. For a full-text version of this topic, go to www.rarediseases.org and click on Rare Disease Database under "Rare Disease Information".

    The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.

    It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report

    This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

    For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org

    Last Updated: 2/24/2012
    Copyright 1987, 1989, 1992, 2002, 2003, 2004, 2007, 2009, 2012 National Organization for Rare Disorders, Inc.

    WebMD Medical Reference from the National Organization for Rare Disorders

    Last Updated: May 28, 2015
    This information is not intended to replace the advice of a doctor. Healthwise disclaims any liability for the decisions you make based on this information.

    Today on WebMD

    child with red rash on cheeks
    What’s that rash?
    plate of fruit and veggies
    How healthy is your child’s diet?
     
    smiling baby
    Treating diarrhea, fever and more.
    Middle school band practice
    Understanding your child’s changing body.
     

    worried kid
    fitArticle
    jennifer aniston
    Slideshow
     
    Measles virus
    Article
    sick child
    Slideshow
     

    babyapp
    New
    Child with adhd
    Slideshow
     
    rl with friends
    fitSlideshow
    Child Coughing or Sneezing into Elbow
    Article