Letterer-Siwe Disease

Medically Reviewed By William C. Lloyd III, MD, FACS
Langerhans cell histiocytosis blood sample
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Letterer-Siwe disease is an outdated name for a cluster of symptoms (a syndrome) caused by Langerhans cell histiocytosis (LCH). Letterer-Siwe disease (pronunciation: “let-ter-er see-vuh” disease) is a rare childhood disease with a poor prognosis.

What is Letterer-Siwe disease?

Letterer-Siwe disease refers to a group of symptoms caused by LCH. Doctors generally do not diagnose Letterer-Siwe disease as a distinct syndrome anymore. Instead they simply diagnose Langerhans cell histiocytosis.

LCH is a disorder that affects the Langerhans cells, which are immune system cells classified as dendrites. As a type of white blood cell, these dendritic cells normally signal the presence of an infection to other immune system cells. Sometimes, genetic mutations in the dendrites cause them to multiply much more rapidly than they should and create cellular deposits in the tissues where they normally reside, such as the skin, mouth, eyes, bone marrow, lungs, and other areas of the body.

Letterer-Siwe disease mainly affects children and usually arises between 2 and 3 years of age, but adults can develop the disorder, as well. Some researchers consider LCH to be a form of cancer, though not all experts agree with this classification.

What are the symptoms of Letterer-Siwe disease?

Langerhans cell histiocytosis symptoms can vary considerably, depending on the area of the body affected. Sometimes, symptoms affect only a limited area, such as the skin. However, Letterer-Siwe disease symptoms affect multiple bodily systems. In a young child, signs and symptoms may include:

  • Ear drainage
  • Enlarged lymph nodes (swollen glands)
  • Enlarged spleen or liver
  • Scaly, sometimes purplish, rash on the scalp, abdomen, ear canals, or skin folds that may look like eczema or seborrhea

What are the causes of Letterer-Siwe disease?

All types of Langerhans cell histiocytosis result from genetic mutations in the Langerhans cells. Researchers have identified four specific genetic mutations that can cause LCH symptoms. These mutations may result from:

  • Having a parent who was exposed to certain chemicals
  • Having a parent who was exposed to workplace dust from granite, metal or wood
  • Having multiple infections during infancy
  • Not being vaccinated as a child

Other risk factors for LCH include:

  • Family history of Letterer-Siwe disease or any syndrome associated with Langerhans cell histiocytosis
  • Family history of cancer
  • Hispanic ancestry
  • Personal or family history of thyroid disease

What are the treatments for Letterer-Siwe disease?

Letterer-Siwe disease treatment always includes supportive care, such as attention to skin hygiene to avoid skin breakdowns that could lead to infection. Other treatments for LCH may vary, depending on the specific body systems and organs involved. Treatments for Letterer-Siwe disease may include:

  • Chemotherapy (to stop the mutant dendrites from multiplying)
  • Corticosteroid injections
  • Hormone replacement therapy
  • Immunotherapy
  • Surgery

Doctors who specialize in hematology and oncology have the medical training to treat patients with Langerhans cell histiocytosis. Pulmonologists and critical care specialists may treat complications of the disorder. However, it is a rare condition so many doctors may not have experience treating it. Contact the Histiocytosis Association for more information or to find a doctor who specializes in LCH. A nationwide search at healthgrades.com using the search term ‘Langerhans cell histiocytosis’ is another option.

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  1. Letterer-Siwe Disease. Merriam-Webster. https://www.merriam-webster.com/medical/Letterer-Siwe%20disease
  2. Langerhans Cell Histiocytosis Treatment (PDQ®)–Patient Version. U.S. National Cancer Institute. https://www.cancer.gov/types/langerhans/patient/langerhans-treatment-pdq
  3. Langerhans Cell Histiocytosis. Merck Manuals, Professional Version. https://www.merckmanuals.com/professional/hematology-and-oncology/histiocytic-syndromes/langerhans-cell-histiocytosis
  4. Letterer-Siwe Disease. Access Anesthesiology. http://accessanesthesiology.mhmedical.com/content.aspx?bookid=852§ionid=49517855
Medical Reviewer: William C. Lloyd III, MD, FACS
Last Review Date: 2020 Sep 2
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