Histiocytoses non-langerhans cell histiocytoses D76.3

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 14.10.2022

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non-Langerhans cell histiocytosis; Non-Langerhans cell histiocytosis

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The non-Langerhans cell histiocytoses are grouped under the name"xanthogranulomas" to show the affiliation of clinically different diseases that show histological similarities to xanthogranulomas.

They are mono- (skin, bone, lymph nodes), more rarely polyorganic, localized or generalized, benign neoplasms due to proliferation of cells of the monocyte-macrophage series. The diagnosis of "non-Langerhans cell histiocytosis" is made by the absence of electron-optical and immunohistological characteristics of Langerhans cells.

With the monoclonal antibody MS-1 (high molecular weight protein) a marker seems to have been found which is strongly expressed in lesional macrophages of non-Langerhans cell histiocytoses. This may suggest that disease patterns such as:

are different morphological expressions of a single disease entity.

Also, the ability or "inability" to store fat, previously listed as a differentiating feature of individual diseases, is more likely to be attributed to different stages of development (example: xanthomatous or non-xanthomatous variant of juvenile xanthogranuloma). The nosological status of histiocytoma (cell-rich dermatofibroma) is as controversial as that of normolipemic xanthomatoses or xanthelasma.

Even in dermatofibromas (old nomenclature: histiocytomas), the storage capacity (e.g., hemosiderin) cannot be taken as an expression of a separate type of tumor. It is rather to be seen as a functional restitution of a cell per se capable of phagocytosis.

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Within the group of non-Langerhans cell histiocytoses(xanthogranuloma family, see Clinical and further classification there), 2 subgroups can be distinguished from a clinical point of view:

  • Systemic non-Langerhans cell histiocytosis: Systemic Langerhans cell histiocytosis is dermatologically irrelevant and is dealt with in pediatric textbooks.
  • Cutaneous non-Langerhans cell histiocytosis:
    • Classical cutaneous non-Langerhans cell histiocytosis (MS-1-)
    • Spindle cell cutaneous non-Langerhans cell histiocytosis (MS-1±).

See Table 1.

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There are no established treatment regimes for cutaneous non-Langerhans cell histiocytosis. In juvenile xanthogranulomas, spontaneous healing must be awaited. S.a. under the respective clinical pictures.

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Quoad vitam good; for the individual clinical pictures see there.

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Classification of cutaneous non-Langerhans cell histiocytosis (varies according to Goerdt)

Classical cutaneous non-Langerhans cell histiocytosis (MS-1-)

Juvenile xanthogranulomas

juvenile xanthogranuloma

benign cephalic histiocytosis

solitary and multicenter reticulohistiocytomas of childhood

papular xanthomatosis

generalized childhood eruptive histiocytomas

Adult xanthogranulomas

adult xanthogranuloma

generalized eruptive histiocytomas

papular xanthomas

multicenter reticulohistiocytosis

Necrobiotic xanthogranulomas

necrobiotic xanthogranuloma with paraproteinemia

Spindle cell cutaneous non-Langerhans cell histiocytosis (MS-1+/-)

Histiocytoma/cell-rich dermatofibroma

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  1. Arico M et al (2003) Histiocytic disorders. Hematol J 4: 171-179
  2. Gianotti R et al (1993) Benign cephalic histiocytosis: a distinct entity or part of a wide spectrum of histiocytic proliferative disorders in children? Am J Dermatopathol 14: 315-319
  3. Goerdt S (1998) Histiocytic tumours. In: Garbe C, Rassner G Dermatology, guidelines and quality assurance for diagnostics and therapy. Springer Verlag, Berlin Heidelberg New York, pp. 372-378
  4. Goerdt S (1993) Inducible expression of MS-1 high molecular weight protein by endothelial cells of continuous origin and by dendritic cells/macrophages in vivo and in vitro. On J Path 142: 1409-1422
  5. Klemke CD et al (2003) Atypical generalized eruptive histiocytosis associated with acute monocytic leukemia. J Am Acad Dermatol 49(5 Suppl): 233-236
  6. 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 Catholic 56: 84-102
  7. Parker F (1996) Normocholesterolemic xanthomatosis. Arch Dermatol 122: 1253-1257
  8. The writing Group of the Histiocyte Society (1987) Histiocytosis syndromes in children. Lancet I: 208-209
  9. Walsh LJ (1991) MS-I sinusoidal endothelial antigen is expressed by factor XIIIa+, 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: 14.10.2022