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cutaneous histiocytoses

Friday 24 October 2003

CUtaneous histiocytoses are a heterogeneous group of disorders that are characterized by the proliferation and accumulation of reactive or neoplastic histiocytes.

Three classes of histiocytoses have been defined:

  • class I, Langerhans cell histiocytosis;
  • class II, non-Langerhans cell histiocytosis without features of malignancy;
  • class III, malignant histiocytic proliferations.

Although the disorders in classes I and II usually have a benign appearance on histology and are commonly non-aggressive and self-healing, some can cause debilitating or even fatal outcomes.


- congenital self-healing histiocytosis (CD1a-, S-100-) (1908575)
- cutaneous langerhans histiocytosis
- cutaneous juvenile xanthogranuloma
- sinus histiocytosis with massive lymphadenopathy
- regressing atypical histiocytosis

- nonfamilial histiocytic dermatoarthritis and familial histiocytic dermatoarthritis
- multiple widespread benign lesions

  • xanthoma disseminatum
  • generalized eruptive histiocytoma
  • nodular non-X histiocytosis
  • various xanthomatous eruptions associated with paraproteinemia


- Newman B, Hu W, Nigro K, Gilliam AC. Aggressive histiocytic disorders that can involve the skin. J Am Acad Dermatol. 2007 Feb;56(2):302-16. PMID: 17097374

- Ringel E, Moschella S. Primary histiocytic dermatoses. Arch Dermatol. 1985 Dec;121(12):1531-41. PMID: 2998286