International Journal of Hematology

, Volume 98, Issue 4, pp 379–381 | Cite as

Erdheim–Chester disease and Schnitzler syndrome: so near, and yet so far

  • Petr SzturzEmail author
  • Libor Hlavatý
  • Jiří Prášek
  • Dana Dvořáková
Images in Hematology
A 56-year-old male presented with diabetes insipidus, non-infectious fever, night sweats, and pain in both lower legs and pelvis. Developing gradually over a period of 9 years, these symptoms were accompanied by increased acute phase markers (C-reactive protein = 63 mg/L, erythrocyte sedimentation rate = 52 mm/h and 72 mm/2 h, fibrinogen = 6.5 g/L). Technetium-99m pyrophosphate bone scintigraphy showed abnormal tracer uptake predominantly in the long bones of the lower extremities, with additional hot spots in the skull, right shoulder, left humerus, and both radial bones (Fig.  1). Positron emission tomography (PET) identified a similar pattern of 18F-fluorodeoxyglucose uptake correlating with osteosclerotic lesions on conventional radiography (X-ray) and computed tomography (CT). Subsequently, histological findings of CD68- and S100-positive, CD1a-negative foamy histiocytes from a bone biopsy were compatible with Erdheim–Chester disease. Moreover, the obtained tissue samples were...


Anakinra Chester Disease Monoclonal Immunoglobulin Urticarial Vasculitis Systemic Lupus Erythematodes 
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Conflict of interest

The authors declare no conflict of interest.


  1. 1.
    Simon A, Asli B, Braun-Falco M, De Koning H, Fermand JP, Grattan C, et al. Schnitzler’s syndrome: diagnosis, treatment, and follow-up. Allergy. 2013;68:562–8.PubMedCrossRefGoogle Scholar
  2. 2.
    Aubert O, Aouba A, Deshayes S, Georgin-Lavialle S, Rieu P, Hermine O. Favorable radiological outcome of skeletal Erdheim–Chester disease involvement with anakinra. Joint Bone Spine. 2013;80:206–7.PubMedCrossRefGoogle Scholar
  3. 3.
    Haroche J, Charlotte F, Arnaud L, von Deimling A, Hélias-Rodzewicz Z, Hervier B, et al. High prevalence of BRAF V600E mutations in Erdheim–Chester disease but not in other non-Langerhans cell histiocytoses. Blood. 2012;120:2700–3.PubMedCrossRefGoogle Scholar

Copyright information

© The Japanese Society of Hematology 2013

Authors and Affiliations

  • Petr Szturz
    • 1
    Email author
  • Libor Hlavatý
    • 2
  • Jiří Prášek
    • 3
  • Dana Dvořáková
    • 1
  1. 1.Department of Internal Medicine, Hematology and Oncology, School of MedicineUniversity Hospital Brno and Masaryk UniversityBrnoCzech Republic
  2. 2.Department of Nuclear MedicineČeské Budějovice Regional HospitalČeské BudějoviceCzech Republic
  3. 3.Clinic of Nuclear Medicine, School of MedicineUniversity Hospital Brno and Masaryk UniversityBrnoCzech Republic

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