title: Hand-Schüller-Christian disease and Erdheim-Chester disease: coexistence and discrepancy.
abstract: 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.
authors.affiliation: Shanghai Clinical Center for Diabetes, Department of Endocrinology and Metabolism, Shanghai Key Laboratory of Diabetes Mellitus, Shanghai Diabetes Institute, Shanghai Jiao Tong University Affiliated Sixth People's Hospital, 600 Yishan Road, Shanghai 200233, China.
authors.forename: Jun
authors.initials: J
authors.lastname: Yin
authors.forename: Feng
authors.initials: F
authors.lastname: Zhang
authors.forename: Huizhen
authors.initials: H
authors.lastname: Zhang
authors.forename: Li
authors.initials: L
authors.lastname: Shen
authors.forename: Qing
authors.initials: Q
authors.lastname: Li
authors.forename: Shundong
authors.initials: S
authors.lastname: Hu
authors.forename: Qinghua
authors.initials: Q
authors.lastname: Tian
authors.forename: Yuqian
authors.initials: Y
authors.lastname: Bao
authors.forename: Weiping
authors.initials: W
authors.lastname: Jia
mesh_terms: D000328:Adult; D000469:Alkaline Phosphatase / Q000097:blood; D003919:Diabetes Insipidus / Q000175:diagnosis / Q000473:pathology; D003937:Diagnosis, Differential; D018450:Disease Progression; D031249:Erdheim-Chester Disease / Q000150:complications / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000473:pathology; D005260:Female; D005500:Follow-Up Studies; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000473:pathology; D006801:Humans; D008297:Male; D010026:Osteosclerosis / Q000175:diagnosis / Q000473:pathology; D011859:Radiography
chemical_list: D000469:Alkaline Phosphatase