MR Findings of Erdheim-Chester Disease

Abstract

Lipoid granulomatosis (Erdheim-Chester disease) is a rare but distinct form of histiocytosis. This disease has characteristic radiologic findings involving the musculoskeletal system that are critical to the diagnosis: symmetric sclerosis of the metaphysis and diaphysis of long bones with relative sparing of the epiphysis as depicted on conventional radiography. However, it is a systemic disease that involves multiple organ systems. This pictorial essay is of a single patient imaged over multiple years, using various pulse sequences with both low and high field strength MR scanners. It depicts many of the characteristic findings encountered in this rare systemic disorder.

Neurological manifestations and neuroradiological presentation of Erdheim-Chester disease: report of 6 cases and systematic review of the literature

“…One patient developed cerebral venous thrombosis [42]. All neurological lesions were hyperintense on T2-weighted MRI images and showed enhancement after gadolinium injection [3,9,20,21,34,57]. Prolonged uptake of gadolinium has been described [33,52].…”

Erdheim-Chester Disease of the Central Nervous System: New Manifestations of a Rare Disease

“…3 Nonspecific bone pain is the most commonly reported symptom. 4 Radiographically, ECD can affect virtually any part of the body, but the most common site is bone, where it manifests as symmetric medullary sclerosis. There is a predilection for the appendicular skeleton, particularly the long bones of the upper and lower extremities.…”

Radiotherapy for Erdheim-Chester disease

“…5,10 But, in nearly half of the patients, there are extraskeletal lesions, including the lung, heart, retroperitoneal space, and central nervous system. [4][5][6]9,10 In addition, ECD occurs more frequently in men and usually affects adults aged 26 to 78 years (average, 53 years). 1,3,5,6,8,10 Differential diagnosis includes Langerhans cell histiocytosis (LCH), storage disease (e.g., Gaucher disease, Niemann-Pick disease), sarcoidosis, lymphoma, and metastases.…”

“…[4][5][6]9,10 In addition, ECD occurs more frequently in men and usually affects adults aged 26 to 78 years (average, 53 years). 1,3,5,6,8,10 Differential diagnosis includes Langerhans cell histiocytosis (LCH), storage disease (e.g., Gaucher disease, Niemann-Pick disease), sarcoidosis, lymphoma, and metastases. [1][2][3][4][5][6][7][8][9] Especially, there may be some clinical overlap between ECD and LCH, but radiographs in ECD often reveal symmetrical osteosclerosis with or without lytic components in the diametaphyses of the long bones, with relative sparing of epiphyses.…”

“…A fi eld size Introduction Erdheim-Chester disease (ECD) is a rare, non-Langerhans form of histiocytosis, with distinctive radiological and pathological features. [1][2][3][4][5][6][7][8][9][10] It differs from such forms of Langerhans cell histiocytosis (LCH) as Hand-Schüller-Christian disease, Letterer-Siwe disease, and eosinophilic granuloma, Magnetic resonance imaging. a Coronal T1-weighted spin-echo image showed diffuse signal loss, while b T2-weighted fast spin-echo displayed heterogenous high and low mixed signals, both symmetrically seen in the diametaphyses of the distal femurs and proximal tibias, with epiphyseal sparing Fig.…”