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. Author manuscript; available in PMC: 2022 Aug 10.
Published in final edited form as: Lancet. 2021 Apr 23;398(10295):157–170. doi: 10.1016/S0140-6736(21)00311-1

Table 2:

Clinical features of histiocytoses with single organ involvement that are often associated with difficult or late diagnosis of histiocytosis

Age Histiocytosis group
Recurrent normolipaemic xanthelasma Adult ECD
Exophthalmos Any age L and R
Diabetes insipidus Any age L
Cerebellar syndrome Any age L
Pachymeningitis Any age R
Testis involvement Adult ECD and R
Painful legs Adult ECD
Vertebra plana Child more often than adult LCH
Recurrent dental losses with cysts Any age LCH
Recurrent pneumothorax Adolescent and young adult LCH
Interstitial lung disease Adult ECD and R
Obstructive renal failure Adult ECD and R

ECD=Erdheim-Chester disease. LCH=Langerhans cell histiocytosis.