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- Author: Periklis Foukas x
- General Orthopaedics x
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Non-Langerhans cell histiocytosis (N-LCH) summarizes a group of rare diseases with different clinical presentations, pathogenesis and morphology. These include primary cutaneous N-LCH, cutaneous N-LCH with systemic involvement, and primary extracutaneous systemic forms with occasional cutaneous involvement.
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The juvenile (JXG) and non-juvenile xanthogranuloma (N-JXG) family of histiocytoses are N-LCH: the JXG family consisting of the JXG (cutaneous), xanthoma disseminatum (cutaneous and systemic) and Erdheim-Chester disease (ECD; systemic); and the N-JXG family consisting of the solitary reticulohistiocytoma (cutaneous), multicentric reticulohistiocytosis (cutaneous and systemic) and Rosai-Dorfman disease (RDD; systemic).
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ECD is a clonal disorder from the JXG family of N-LCH; RDD is a reactive proliferative entity from the non-juvenile xanthogranuloma family of N-LCH.
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ECD and RDD N-LCH are rare disorders, which are difficult to diagnose, with multi-organ involvement including bone and systemic symptoms, and which respond to therapy in an unpredictable way.
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The key to successful therapy is accurate identification at tissue level and appropriate staging. Patients should be observed and monitored in a long-term pattern. Prognosis depends on disease extent and the organs involved; it is generally good for RDD disease and variable for ECD.
Cite this article: EFORT Open Rev 2018;3:381-390. DOI: 10.1302/2058-5241.3.170047