• Sunil K. KothiwalaEmail author
  • Piyush KumarEmail author
  • Rajesh Kumar MandalEmail author
Living reference work entry


The histiocytoses are a group of disorders characterized by accumulation of variable number of monocytes, macrophages, and dendritic cells in affected tissues of children and adults. Many different subtypes have been described with a wide range of clinical features and histopathological changes.

Langerhans cell histiocytoses include spectrum of clinical manifestation ranging from self-healing lesions to life-threatening disseminated disease. Juvenile xanthogranuloma is the most common type of non-Langerhans cells histiocytoses, and some other subtypes may show overlapping features. Necrobiotic xanthogranuloma, Rosai-Dorfman disease, Erdheim-Chester disease, and multicentric reticulohistiocytosis are characterized by systemic involvement. Infiltrating cells of malignant histiocytoses show cancerous changes. The diagnosis of histiocytoses is based on clinical findings in combination with histopathological analysis identifying tissue infiltration by histiocytes with ultrastructural and immunophenotypic characteristics of cells. Various different chemotherapeutic agents, lasers, cryotherapy, and radiofrequency ablation have shown variable success in the treatment of histiocytoses.


Histiocyte Birbeck granule CD1a S100 Langerin CD68 Factor Fascin Histiocytosis Classification Langerhans cell histiocytosis Coffee-bean shape Reniform pattern Hashimoto-Pritzker disease Eosinophilic granuloma Letterer-Siwe disease Hand-Schüller-Christian disease Congenital self-healing reticulohistiocytosis Histiocytosis X Langerhans cell Non-Langerhans cell histiocytosis Juvenile xanthogranuloma Xanthogranulomatous reaction Benign cephalic histiocytosis Generalized eruptive histiocytosis Popular xanthoma Progressive nodular histiocytosis Factor XIIIa positive histiocytosis Plane xanthoma Diffuse normolipemic plane xanthomatosis Paraproteinemia Hereditary progressive mucinous histiocytosis Xanthoma disseminatum Xanthogranuloma Multicentric reticulohistiocytosis Polyarthritis Rosai-Dorfman Emperipolesis Erdheim-Chester Familial sea blue histiocytosis May-Grunwald stain Neimann-Pick disease Malignant histiocytoses True histiocytic lymphoma Histiocytic sarcoma Monocytic leukemia Malignant histiocytosis Histiocytic lymphoma Lymphoma Malignant histiocyte Sarcoma 


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© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Consultant Dermatologist, SkinEva ClinicJaipurIndia
  2. 2.Department of DermatologyKatihar Medical CollegeKatiharIndia
  3. 3.North Bengal Medical CollegeDarjeelingIndia

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