Unusual variants of non-Langerhans cell histiocytoses

J Am Acad Dermatol. 2007 Dec;57(6):1031-45. doi: 10.1016/j.jaad.2007.03.014. Epub 2007 May 7.


Histiocytic syndromes represent a large, heterogeneous group of diseases resulting from proliferation of histiocytes. In addition to the classic variants, the subset of non-Langerhans cell histiocytoses comprises rare entities that have more recently been described. These last include both forms that affect only the skin or the skin and mucous membranes, and usually show a benign clinical behavior, and forms involving also internal organs, which may follow an aggressive course. The goal of this review is to outline the clinical, histologic, and ultrastructural features and the course, prognosis, and management of these unusual histiocytic syndromes.

Publication types

  • Review

MeSH terms

  • Adult
  • Child
  • Diagnosis, Differential
  • Histiocytosis, Non-Langerhans-Cell* / classification
  • Histiocytosis, Non-Langerhans-Cell* / diagnosis
  • Histiocytosis, Non-Langerhans-Cell* / pathology
  • Histiocytosis, Non-Langerhans-Cell* / therapy
  • Humans