Erdheim-Chester disease in pictures


Authors: P. Szturz 1;  Z. Adam 1;  R. Koukalová 2;  Z. Řehák 2;  J. Neubauer 3;  J. Prášek 4;  M. Doubek 1;  R. Hájek 1;  J. Mayer 1
Authors‘ workplace: Interní hematoonkologická klinika LF MU a FN Brno, pracoviště Bohunice, přednosta prof. MUDr. Jiří Mayer, CSc. 1;  Oddělení nukleární medicíny, PET centrum Masarykova onkologického ústavu Brno, přednosta prim. MUDr. Karol Bolčák 2;  Radiologická klinika Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednosta prof. MUDr. Vlastimil A. Válek, CSc. 3;  Klinika nukleární medicíny Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednosta doc. MUDr. Jiří Prášek, CSc. 4
Published in: Vnitř Lék 2010; 56(Supplementum 2): 170-178
Category: Langerhans cell histiocytosis and some other Hematology rare diseases

Overview

Erdheim-Chester disease (ECD) characterized by proliferation of foamy histiocytes and their infiltration into various tissues and organs, typically the long bones of the lower extremities, is a rare disease of the non-Langerhans cell histiocytosis group. In the patients with ECD during examinations by imaging methods various pathologic changes are described, including osteosclerosis, infiltration of the hypophysis, periaortic fibrosis (coated aorta) as well as retroperitoneal fibrosis reminding of Ormond’s disease. In this work we are presenting our collection of 83 images depicting these and many other interesting findings arranged into 17 pictures. Published here are images obtained by conventional radiography (CR), computed tomography (CT), magnetic resonance imaging (MRI), traditional bone scintigraphy, single photon emission computed tomography (SPECT) and also by means of positron emission tomography (PET) or hybrid PET/CT imaging if need be. In conclusion, we underline the importance of whole-body PET/CT acquisition, including lower limbs, which depicts typical bilateral symmetric osteosclerosis of the long bones with diffusely increased radiopharmacon (fluorodeoxyglucose) uptake.

Key words:
Erdheim-Chester disease – osteosclerosis – diabetes insipidus centralis – periaortic fibrosis – retroperitoneal fibrosis – conventional radiography – CT – MR – skeletal scintigraphy – SPECT – PET/CT


Sources

1. Chester W. Über Lipoidgranuolmatose. Virchows Arch 1930; 279: 561–602.

2. Jaffe HL. Metabolic, degenerative and inflammatory disease of bones and joints. Philadelphia: PA Lea and Febiger 1972: 531–541.

3. Adam Z, Balšíková K, Pour L et al. Diabetes insipidus, následovaný po 4 letech dysartrií a lehkou pravostrannou hemiparézou – první klinické příznaky Erdheimovy-Chesterovy nemoci. Popis a zobrazení případu s přehledem informací o této nemoci. Vnitř Lék 2009; 55: 1173–1188.

4. Adam Z, Krejčí M, Vorlíček J. Hematologie: přehled maligních hematologických nemocí. Praha: Grada Publishing 2008.

5. Arnaud L, Malek Z, Archambaud F et al. 18F-fluorodeoxyglucose-positron emission tomography scanning is more useful in follow up than in the initial assessment of patients with Erdheim-Chester disease. Arthritis Rheum 2009; 60: 3128–3138.

6. Loeffler AG, Memoli VA. Myocardial involvement in Erdheim-Chester disease. Arch Pathol Lab Med 2004; 128: 682–685.

7. Lachenal F, Cotton F, Desmurs-Clavel H et al. Neurological manifestations and neuroradiological presentation of Erdheim-Chester disease: report of 6 cases and systematic review of the literature. J Neurol 2006; 253: 1267–1277.

8. Veyssier-Belot C, Cacoub P, Caparros-Lefebvre D et al. Erdheim-Chester disease. Clinical and radiologic characteristics of 59 cases. Medicine (Baltimore) 1996; 75: 157–169.

9. Karcioglu ZA, Sharara N, Boles TL et al. Orbital xanthogranuloma: clinical and morphologic features in eight patients. Ophthal Plast Reconstr Surg 2003; 19: 372–381.

10. Tritos NA, Weinrib S, Kaye TB. Endocrine manifestations of Erdheim-Chester disease (a distinct form of histiocytosis). J Intern Med 1998; 244: 529–535.

11. Sheu SY, Wenzel RR, Kersting C et al. Erdheim-Chester disease: case report with multisystemic manifestations including testes, thyroid, and lymph nodes, and a review of literature. J Clin Pathol 2004; 57: 1225–1228.

12. Sheen KC, Chang CC, Chang TC et al. Thickened pituitary stalk with central diabetes insipidus: report of three cases. J Formos Med Assoc 2001; 100: 198–204.

13. Bangard C, Lotz J, Rosenthal H et al. Erdheim-Chester disease versus multifocal fibrosis and Ormond’s disease: a diagnostic dilemma. Clin Radiol 2004; 59: 1136–1141.

14. Haroche J, Amoura Z, Touraine P et al. Bilateral adrenal infiltration in Erdheim-Chester disease. Report of seven cases and literature review. J Clin Endocrinol Metab 2007; 92: 2007–2012.

15. Droupy S, Attias D, Eschwege P et al. Bilateral hydronephrosis in a patient with Erdheim-Chester disease. J Urol 1999; 162: 2084–2085.

16. O’Rourke R, Wong DC, Fleming S et al. Erdheim-Chester disease: a rare cause of acute renal failure. Australas Radiol. 2007; 51: B48–B51.

17. Gupta A, Aman K, Al-Babtain M et al. Multisystem Erdheim-Chester disease; a unique presentation with liver and axial skeletal involvement. Br J Haematol 2007; 138: 280.

18. Krüger S, Krop C, Wibmer T et al. Erdheim-Chester disease: a rare cause of interstitial lung disease. Med Klin (Munich) 2006; 101: 573–576.

19. Vaglio A, Corradi D, Maestri R et al. Pericarditis heralding Erdheim-Chester disease. Circulation 2008; 118: e511–e512.

20. Serratrice J, Granel B, De Roux C et al. “Coated aorta”: a new sign of Erdheim-Chester disease. J Rheumatol 2000; 27: 1550–1553.

21. Kudo Y, Iguchi N, Sumiyoshi T et al. Dramatic change of Ga-67 citrate uptake before and after corticosteroid therapy in a case of cardiac histiocytosis (Erdheim-Chester disease). J Nucl Cardiol 2006; 13: 867–869.

22. Dion E, Graef C, Miquel A et al. Bone involvement in Erdheim-Chester disease: imaging findings including periostitis and partial epiphyseal involvement. Radiology 2006; 238: 632–639.

Labels
Diabetology Endocrinology Internal medicine

Article was published in

Internal Medicine

Issue Supplementum 2

2010 Issue Supplementum 2

Most read in this issue

This topic is also in:


Login
Forgotten password

Don‘t have an account?  Create new account

Forgotten password

Enter the email address that you registered with. We will send you instructions on how to set a new password.

Login

Don‘t have an account?  Create new account