Histiocytoses D76.3

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 08.05.2023

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benign histiocytosis

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In the classification adopted in 1987 (Working Group of the Histiocyte Society), histiocytoses were divided into:

  • Langerhans cell histiocytoses
  • Non-Langerhans cell histiocytoses
  • Malignant histiocytoses.

This classification took into account morphological and functional findings on macrophages, dendritic cellsand monocytes.

The expanded classification of histiocytoses published in 2016 (Emile JF et al 2016) is based on clinical, radiological, phenotypic, genetic, and/or molecular features. In this classification, histiocytoses are divided into 5 groups, each of which can be assigned definable entities.

  • L group - Langerhans cell group
  • C-group - Cutaneous non-Langerhans cell histiocytoses
  • R group -Rosai-Dorfman disease
  • M group -Malignant histiocytoses
  • H group - Hemophagocytic lymphohistiocytosis and macrophage activation syndromes

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Mono- or polyorganic, localized or disseminated (also generalized), benign or malignant neoplasms caused by the proliferation of cells of the monocytic-phagocytic system (MPS/ see below histiocytes). To the monocytic-phagocytic system belong the dendritic cells which occur in various organs. Their main task is the antigen presentation to naive T-lymphocytes. This is done by their MHC molecules (major histocompatibility complex).

The representatives of these specialized dendritic cells in multilayered squamous epithelia are the Langerhans cells. Langerhans cells express CD1a as well as langerin (CD207) and exhibit the characteristic Bierbeck granules. They can be well characterized by this particular profile.

After activation, they migrate to the regional lymph nodes where they transform into interdigitating dendritic cells.

All dendritic cells, like monocytes and macrophages, are derived from pluripotent bone marrow cells.

Histiocytoses occur with varying clinic in all age groups.

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Classification of histiocytoses (Emile JF et al. 2016).

L-group (Langerhans cell group/s.u. Langerhans cell histiocytoses)

C group (Cutaneous non-Langerhans cell histiocytoses/cutaneousnon-Langerhans cell histiocytoses with/without significant systemic involvement)

Xanthogranuloma (XG) - family (see below Xanthogranulomas )

Non-xanthogranuloma family

R group (Rosai-Dorfman group)

M group (malignant histiocytoses; very rare).

  • Primary malignant histiocytoses (manifest primarily in the skin)
  • Secondary malignant histiocytoses (hematologic neoplasms diagnosed hematologically with secondary skin involvement) - Langerhans cell sarcoma

H-group (hemophagocytic lymphohistiocytoses)


Instead of the previously listed classification according to entities (probably these are not entities but only different manifestations and courses of a single underlying disease), a stratification for the classical Langerhans cell histiocytoses has proven useful, especially also for therapy studies. This classification includes the involvement of organs at risk(liver, spleen, bone marrow).

Single-system disease:

  • Monoorganic solitary involvement: skin/unilocular skeletal involvement/solitary lymph node/LCH of the lung.
  • Monoorganic multiple infestation: e.g. multilocular skeletal infestation/infestation of multiple lymph nodes.

Multi-system disease (involvement of multiple organ systems including skin (old name: Abt-Letterer-Siwe disease, Hand-Schüller-Christian disease).

  • Low risk: patient > 2 years; no involvement of the hematopoietic system, liver, or spleen
  • High risk: patient < 2 years, or patient > 2 years with involvement of the organs at risk (hematopoietic system, liver, spleen).

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See below the respective crakheitsbilder.

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Although related by name, eosinophilic histiocytosis does not belong to histiocytosis, but is a special form of lymphomatoid papulosis.

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  1. Becker G (2003) Multimodal treatment strategy for Langerhans cell histiocytosis at head and neck manifestations. ENT 51: 55-60
  2. Emile JF et al (2016) Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages. Blood 127: 262-281
  3. Goerdt S (1998) Histiocytic tumors. In: Garbe C, Rassner G Dermatology, guidelines and quality assurance for diagnosis and therapy. Springer Verlag, Berlin Heidelberg New York pp 372-378.
  4. Kilborn TN et al (2003) Paediatric manifestations of Langerhans cell histiocytosis: a review of the clinical and radiological findings. Clin Radiol 58: 269-278
  5. Lichtenstein L (1953) Histiocytosis X. Integration of eosinophilic granuloma of bone, Letterer-Siwe Disease, and Schüller-Christian Disease as related manifestations of a single nosologic entity. Arch Pathol 56: 84-102
  6. Ruzicka T et al (2003) Clinical course and therapy of Langerhans cell histiocytosis in children and Adults. Dermatologist 54: 148-155
  7. Scheker LE (2003) Neonatal lupus erythematosus mimicking langerhans cell histiocytosis. Pediatr Dermatol 20: 164-166
  8. Singh A et al (2003) Adult langerhans cell histiocytosis limited to the skin. Dermatology 207: 157-161
  9. Utiikal J et al (2003) The cutaneous non-Langerhans cell histiocytoses. JDDG 1: 471-491
  10. Walsh LJ (1991) MS-I sinusoidal endothelial antigen is expressed by factorXIIIa+, HLA-DR+ dermal perivascular dendritic cells. Lab Invest 65: 732-741


Please ask your physician for a reliable diagnosis. This website is only meant as a reference.


Last updated on: 08.05.2023