• Augusto VaglioEmail author
  • Rossana Rocco
  • Julien Haroche
  • Jean-François Emile


Histiocytoses are rare, often systemic diseases hallmarked by tissue infiltration by abnormal histiocytes bearing peculiar morphological and immunohistochemical characteristics. They are classified based on cell type (e.g. Langerhans cell histiocytosis (LCH) and non-Langerhans forms such as Erdheim-Chester disease (ECD)), preferential organ involvement (e.g. cutaneous forms) and clinical aggressiveness (e.g. malignant histiocytoses). Some histiocytoses are clearly clonal disorders, often driven by somatic mutations of proto-oncogenes, whereas others can be reactive to or associated with systemic immune-mediated or infectious diseases. This chapter will mainly focus on primary systemic histiocytoses such as LCH and ECD and will discuss the role of infections in these complex diseases.


Histiocytosis Erdheim-Chester BRAF Langerhans 



Central nervous system


Cytotoxic T cells


Erdheim-Chester disease


Human herpesvirus 6


Human immunodeficiency virus


Haemophagocytic lymphohistiocytosis








Langerhans cell histiocytosis


Magnetic resonance imaging


Mammalian target of rapamycin


Natural killer


Positron emission tomography


Rosai-Dorfman disease


T-helper 1


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Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  • Augusto Vaglio
    • 1
    Email author
  • Rossana Rocco
    • 1
  • Julien Haroche
    • 2
    • 3
  • Jean-François Emile
    • 4
  1. 1.Nephrology UnitUniversity HospitalParmaItaly
  2. 2.Department of Internal Medicine, French Reference Center for Rare Auto-immune and Systemic DiseasesInstitut E3M, AP-HP, Pitié-Salpêtrière HospitalParisFrance
  3. 3.Université Pierre et Marie Curie, University Paris 6ParisFrance
  4. 4.EA4340 and Pathology DepartmentVersailles University and Ambroise Paré HospitalBoulogneFrance

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