Revised classification of histiocytosis and neoplasms of the macrophage-dendritic cell lineage
| Group | Disease |
|---|---|
| L Group | Langerhans cell histiocytosis (LCH) |
| Indeterminate cell histiocytosis (ICH) | |
| Erdheim-Chester disease (ECD) | |
| Mixed LCH/ECD | |
| C Group | Cutaneous non-LCH |
| - XG family: JXG, AXG, SRH, BCH, GEH, PNH | |
| - N-XG family: cutaneous RDD, NXG, other NOS | |
| Cutaneous non-LCH with major systemic component | |
| R group | Familial Rosai-Dorfman Disease (RDD) |
| Sporadic RDD | |
| - Classical RDD | |
| - Extra-nodal RDD | |
| - RDD with neoplasia or immune disease | |
| - Unclassified | |
| M group | Primary malignant histiocytosis |
| Secondary malignant histiocytosis | |
| H group | Primary hemophagocytic lymphohistiocytosis (HLH) |
| Secondary HLH | |
| HLH of unknown/uncertain origin |
AXG, adult xanthogranuloma; BCH, benign cephalic histiocytosis; GEH, generalized eruptive histiocytosis; JXG, juvenile xanthogranuloma; NXG, necrobiotic xanthogranuloma; PNH, progressive nodular histiocytosis; SRH, solitary reticulohistiocytoma. Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages [5].