Two Enlarged Kidneys: A Manifestation of Erdheim-Chester Disease

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Abstract

Erdheim-Chester disease is a rare form of non-Langerhans’ cell histiocytosis. This disorder is characterized by a bone involvement and several extraskeletal manifestations. We describe the case of a patient with a pleural and pericardial effusion leading to tamponade. Pathological examination of pericardium and mediastinal adenopathy was normal. The abdominal computed tomography scan showed two enlarged kidneys suggestive of Erdheim-Chester disease. Bone scan scintigraphy demonstrated symmetrical increased labeling of the long bones. The biopsy of perirenal soft tissue confirmed the diagnosis of Erdheim-Chester disease.

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Erdheim-Chester disease. Clinical and radiologic characteristics of 59 cases.

Géraldine H Petit, B Wallaert, P Cacoub … (1996)

We made a retrospective evaluation of clinical and radiologic features, treatment, and outcome of Erdheim-Chester disease, a rare non-Langerhans cell histiocytosis. We had 7 patients coming from 3 French teaching hospitals and reviewed 52 cases from the literature. These cases were considered to have Erdheim-Chester disease when they had either typical bone radiographs (symmetrical long bones osteosclerosis) and/or histologic criteria disclosing histiocytic infiltration without features for Langerhans cell histiocytosis (no S-100 protein, no intracytoplasmic Birbeck granules). Ages at diagnosis ranged from 7 to 84 years (mean +/- SD = 53 +/- 14 yr) with a male/female ratio of 33/26. Bone pain was the most frequent clinical sign (28/59), mostly located in the lower limbs. Exophthalmos and diabetes insipidus were found in respectively 16/59 and 17/59 patients. General symptoms (fever, weight loss) and "xanthomas" (mainly located on the eyelids) were present in 11/59 patients. Retroperitoneal involvement was found in 17/59 patients. Skeletal X-ray showed typical osteosclerosis of the diaphysis of the long bones in 45/59 patients. Bone radiographs showed osteolytic lesions of the flat bones (skull, ribs) in 8 patients. Histologic diagnosis was performed after a bone biopsy (28 patients), a retroorbital biopsy (9 patients), and/or a biopsy of the retroperitoneal infiltration or the kidney (11 patients). Six of our 7 patients but only 5 of 52 patients from the literature had the complete histologic criteria, disclosing no Birbeck granules or S-100 immunostaining. In other cases, histologic results usually described a xanthogranulomatous infiltration by foamy histiocytes nested in fibrosis. Treatment was corticotherapy (20/59), chemotherapy (8/59), radiotherapy (6/59), surgery (3/59) and immunotherapy (1 patient). Twenty-two patients died after a mean follow-up of 32 +/- 30 mo (range, 3-120 mo). In conclusion, Erdheim-Chester disease may be confused with Langerhans cell histiocytosis as it sometimes shares the same clinical (exophthalmos, diabetes insipidus) or radiologic (osteolytic lesions) findings. However, it also appears to have distinctive features. Patients are older and have a worse prognosis than those with Langerhans cell histiocytosis, and the diagnosis relies on the association of specific radiologic and histologic findings.

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Erdheim-Chester Disease

A. Egan, Lisa A Boardman, Henry Tazelaar … (1999)

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The role of bone scintigraphy in patients with Erdheim-Chester disease.

Suresh K. Joseph, P Barth, William D. Brandt … (2000)

Erdheim-Chester disease (ECD) is a rare disorder that has been reported fewer than 60 times in the literature. Although clinical findings seem to be specific at first sight, histologic classification remains unclear. It has not been decided whether ECD is part of the spectrum of histiocytoses or whether it may be a lipid storage disorder or even a primary macrophage cell disorder, although it does show a distinct histologic pattern. However, the clinical appearance alone shows several typical features, rendering the diagnosis very probable if present. This article illustrates the importance of bone scanning in ECD, because the scintigraphic pattern of involved skeletal sites may in themselves lead to the diagnosis. Several differential diagnoses are considered. The importance of bone scintigraphy as an imaging method in patients with an unclear diagnosis is discussed, as exemplary in ECD, as is its role for the detection of sites of skeletal involvement in other diseases.

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Journal

Journal ID (publisher-id): AJN

Journal ID (nlm-ta): Am J Nephrol

Journal ID (doi): 10.1159/issn.0250-8095

Title: American Journal of Nephrology

Publisher: S. Karger AG

ISSN (Print): 0250-8095

ISSN (Electronic): 1421-9670

Publication date Subscription-year: 2001

Publication date (Print): August 2001

Publication date (Electronic): 13 August 2001

Volume: 21

Issue: 4

Pages: 315-317

Affiliations

Departments of ^aInternal Medicine, ^bRadiology, ^cPathology and ^dCardiology, Groupe Hospitalier Saint-Jacques, Clermont-Ferrand and ^eDepartment of Internal Medicine, Pitié-Salpêtrière Hospital, Paris, France

Article

Publisher ID: 46267 Other ID: Am J Nephrol 2001;21:315–317

DOI: 10.1159/000046267

PubMed ID: 11509804

SO-VID: 4d51fe81-f4c8-46c7-8a3a-52cc9ca47f55

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Two Enlarged Kidneys: A Manifestation of Erdheim-Chester Disease

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Karger: Nephrology

Most cited references 3

Erdheim-Chester disease. Clinical and radiologic characteristics of 59 cases.

Erdheim-Chester Disease

The role of bone scintigraphy in patients with Erdheim-Chester disease.

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