MULTISYSTEM NON‐ARTHROPATHIC RETICULOHISTIOCYTOSIS Problems and pitfalls in the differential diagnosis of multisystem non‐Langerhans cell histiocytoses

Abstract

Histiocytoses are rare proliferative disorders of the mononuclear‐phagocyte system. Within non‐Langerhans‐cell histiocytoses (NLCH), multisystem (MS) cases potentially constitute a subset at high risk of complications and progression. The current 2016 revised classification subdivides histiocytoses in 5 groups, based on highly heterogeneous criteria. Within MS‐NLCH, the "L‐group" includes Erdheim‐Chester disease (ECD) together with Langerhans‐cell histiocytosis, due to their molecular similarity; disseminated juvenile xanthogranuloma, xanthoma disseminatum and multicentric reticulohistiocytosis belong to the "C‐group", because of their predominant dermatological presentation; and lastly, MS‐Rosai‐Dorfman disease (RDD) is listed in the specific "R‐group" due to clinical and pathological findings. However, from a histopathological point of view, MS‐NLCH may be divided into three categories: foamy‐cell NLCH (e.g. ECD), Reticulohistiocytoses and RDD.

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