Histiocytosis 4/5 (4)

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Periorbital tumor of Langerhans histiocytosis. Via Wikipedia. Original from University of Cincinnati. CC BY 2.5.


Histiocytosis is a group of rare diseases there histiocytes (special white blood cells: tissue macrophages) by mistake are stored in large quantities in various organs. Histiocytosis was previously (WHO 1987) divided into three main groups:

  1. Langerhans histiocytosis
  2. Non-Langerhans 
  3. Malignant (malignant) histiocytosis

Later, it is proposed to be divided into five main groups (reference: Emile JF, 2016).

  • Older names of the diseases are Histiocytosis-X, Letter-Seven disease, Hand-Chüller-Christian disease and diffuse reticulo-endotheliosis
  • Tissue samples (biopsy) are sometimes described by histiocytosis as «eosinophilic granuloma»


Histiocytosis is defined as one rare disease.


Symptoms and findings vary.


The diagnosis is made on the basis of symptoms and investigation findings. One uses CT-MR and PET / CT examinations, but the result of tissue test (biopsy) is crucial.

1. »L» (Langerhans) group

  • Langerhans cell histiocytosis

    Langerhans Histiocytosis Swelling of the head / scalp. Failla V, Rare Tumors (2010). Open

    • Symptoms and severity vary from harmless to severe
    • 76% are children under the age of 10
    • Fever
    • Weight Loss
    • Multiple skeletal swelling, most often in the head / skull
    • Eczema and wounds, most often on the head (80%)
    • Hepatosplenomegaly (large liver and spleen) and lymph node enlargements
    • Lungs with asymptomatic, nodular opacities (pulmonary histiocytosis X)
  • Erdheim-Chester disease

  • Erdheim-Chester, histiocytosis with skeletal changes close to the ankle joint. Adawi M, Mdicine (Baltimore) 2016, CC BY-NC-ND 4.0


  • Juvenile xanthogranuloma with manifestation beyond the skin

    • Skin abnormalities
    • Nervous system (CNS), liver and spleen, eye, throat, muscles
    • Most often children under the age of 1, but can also be seen in older children and adults

2. «C» group (Cutan (skin) and mucocutane (skin and mucous membranes)

Histiocytosis in skin. Bakery OA, Rare Tumors (2013).

  • Reticulo-histiocytosis (restricted and multisentric type); please read more here (in Danish)

    • Multisentric type
      • Severe arthritis (joint inflammation) in hands
      • Widespread skin and significant Arthritis-changes
      • Usually women 50-60 years
      • Varying, swinging disease activity over several years
  • Xantogranulom group
    • Different forms that can attack from young children to adults
    • Some or many red-yellow nodules in the skin
    • 0,5-1,0 cm in diameter
    • If other organs are also included, the disease is classified in the «L» Group (see above)
  • Sea-blue histiocytosis

    • Skin affection with histiocytes in adipose tissue
    • Hereditary or after prolonged parenteral fatty nutrition

3. «M» (Malignant / Cancer) Group. Malignant histiocytosis

  • Symptoms: Cough, decreased appetite, anemia, dyspnoea
  • Lungs, lymph nodes, liver, spleen and CNS may be affected by histiocytic infiltration (biopsy)
  • Various primary forms that are separated from lymphoma by a tissue sample
  • Secondary forms of lymphoma and leukemia

4. "R" (Rosai-Dorfman) group and various forms that do not attack skin

5. «H» Group. Hemophagocytic Lymphohistiocytosis (HLH) og Macrophage activation syndrome (MAS)


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