Open AccessHand‐Schüller‐Christian Disease and Erdheim‐Chester Disease: Coexistence and Discrepancy
Author(s) -
Yin Jun,
Zhang Feng,
Zhang Huizhen,
Shen Li,
Li Qing,
Hu Shundong,
Tian Qinghua,
Bao Yuqian,
Jia Weiping
Publication year - 2013
Publication title -
the oncologist
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.176
H-Index - 164
eISSN - 1549-490X
pISSN - 1083-7159
DOI - 10.1634/theoncologist.2012-0234
Subject(s) - medicine , diabetes insipidus , erdheim–chester disease , histiocytosis , osteosclerosis , pituitary stalk , langerhans cell histiocytosis , pathology , magnetic resonance imaging , disease , radiology , pituitary gland , hormone
CME Learning ObjectivesDistinguish Erdheim‐Chester disease from Langerhans cell histiocytosis. Cite the keys to diagnosis of Hand‐Schüller‐Christian disease in a patient with only central diabetes insipidus. List the signs linking a Hand‐Schüller‐Christian disease patient to coexisting ECD.Langerhans cell histiocytosis (LCH) and Erdheim‐Chester disease (ECD) share similar clinical features and mechanisms. In very rare circumstances, the two diseases coexist in the same patient. Here we report such a patient, who was first diagnosed with Hand‐Schüller‐Christian disease (HSC), a type of LCH. Several years later, the patient presented with severe exophthalmos and osteosclerosis on radiograph. New biopsy revealed ECD. We also analyze 54 cases of LCH and 6 cases of ECD diagnosed in our hospital, as well as their progression during a follow‐up period of 8 years. In five cases of HSC (9.3% of LCH), a triad of central diabetes insipidus, hyperprolactinemia, and pituitary stalk thickening on magnetic resonance imaging (MRI) preceded the typical bone lesions by 4–9 years. In addition, LCH was featured as elevated plasma alkaline phosphatase (ALP), which was normal in ECD. Combined with a literature review, several features are summarized to differentiate ECD from HSC. In patients with diabetes insipidus, concomitant hyperprolactinemia and pituitary stalk thickening on MRI indicate a possible HSC. Additionally, if osteosclerosis is observed in a patient with LCH, the coexistence of ECD should be considered.