Hand-Schüller-Christian disease and Erdheim-Chester disease: coexistence and discrepancy.


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.

DOI: 10.1634/theoncologist.2012-0234

Cite this paper

@article{Yin2013HandSchllerChristianDA, title={Hand-Sch{\"{u}ller-Christian disease and Erdheim-Chester disease: coexistence and discrepancy.}, author={Jun Yin and Feng Zhang and Huizhen Zhang and Li Shen and Qing Li and Shundong Hu and Qinghua Tian and Yuqian Bao and Weiping Jia}, journal={The oncologist}, year={2013}, volume={18 1}, pages={19-24} }