Pulmonary involvement in Erdheim‐Chester disease

Chung, Jae Ho; Park, Moo Suk; Shin, Dong Hyun; Choe, Kyu Ok; Kim, Se K.; Chang, Joon; Kim, Sung K.; Kim, Young S.

doi:10.1111/j.1440-1843.2005.00671.x

Cited by 27 publications

(5 citation statements)

References 13 publications

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“…One of the main clinical features of ECD is pain secondary to bilateral osteosclerosis of the long bones 40,46–48 . Extraskeletal manifestations are seen in over half of the cases and can occur in almost any organ including the lungs, heart, skin, kidneys, retroperitoneum and orbits 49–59 . Cutaneous findings include pruritic rash, xanthelasma and periorbital xanthoma 54,60,61 .…”

Section: Discussionmentioning

confidence: 99%

Non-Langerhans cell histiocytosis with isolated CNS involvement: An unusual variant of Erdheim-Chester disease

Conley¹,

Manjila²,

Guan

et al. 2010

Neuropathology

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Benign histiocytic proliferations are identified by their component cells and classified as either Langerhans cell histiocytosis or non-Langerhans cell histiocytosis. We report a 58-year-old Caucasian woman who presented with diabetes insipidus and was found to harbor a large suprasellar mass. Histopathological analysis was consistent with non-LCH. The differential diagnoses included juvenile xanthogranuloma, adult-onset xanthogranuloma, xanthoma disseminatum, Rosai-Dorfman disease, and Erdheim-Chester disease. Immunohistochemical examination demonstrated a proliferation of large lipid-laden histiocytic cells which were positive for CD68, negative for S100 protein, and showed only faint, background staining for CD1a. We present a case of an autopsy-confirmed non-Langerhans cell histiocytosis limited to the central nervous system and evaluated with both immunohistochemical and ultrastructural studies. Based on the multifocality, anatomic distribution, and immunostaining features, a diagnosis of Erdheim-Chester disease was made. This is only the second reported case of Erdheim-Chester disease with intracranial involvement but absence of extracerebral manifestations. Given the overlapping clinicopathologic, radiographic, and immunohistochemical profiles, differentiating between these rare histiocytic disorders can often present a significant diagnostic challenge. A systematic approach using all available clinical, laboratory, radiographic, histologic, immunohistochemical and ultrastructural data is essential for proper discrimination between the numerous histiocytoses.

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Section: Discussionmentioning

confidence: 99%

Non-Langerhans cell histiocytosis with isolated CNS involvement: An unusual variant of Erdheim-Chester disease

Conley¹,

Manjila²,

Guan

et al. 2010

Neuropathology

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“…The lung infiltrate in patients with ECD usually has a lymphangitic distribution and is concentrated in the interlobular septa and the peribronchovascular and subpleural interstitium (4,8,(31)(32)(33)(34). Unlike Langerhans cells, the histiocytes have abundant pale cytoplasm and do not show nuclear folding, eosinophilic cytoplasm, or Birbek granules.…”

Section: Erdheim-chester Diseasementioning

confidence: 99%

Histiocytic Disorders of the Chest: Imaging Findings

Ahuja¹,

Kanne²,

Meyer³

et al. 2015

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Histiocytic disorders of the chest comprise a broad spectrum of diseases. The lungs may be involved in isolation or as part of systemic disease. Some of these disorders are primary and have unknown etiology, and others result from a histiocytic response to a known cause. Among primary histiocytic disorders, pulmonary Langerhans cell histiocytosis (PLCH) is the most common; others include Erdheim-Chester disease and Rosai-Dorfman disease. Adult PLCH occurs almost exclusively in adults aged 20-40 years who smoke. Pediatric PLCH is extremely rare and typically occurs as part of multisystemic disease. Erdheim-Chester disease affects middle-aged and older adults; thoracic involvement usually occurs as part of systemic disease. Rosai-Dorfman disease affects children and young adults and manifests as painless cervical lymphadenopathy. Examples of secondary histiocytic disorders are storage diseases such as Gaucher disease, Niemann-Pick disease, and Fabry disease; pneumoconiosis such as silicosis and coal workers' pneumoconiosis; and infections such as Whipple disease and malakoplakia. These disorders are characterized at histopathologic examination on the basis of infiltration of alveoli or the pulmonary interstitium by histiocytes, which are a group of cells that includes macrophages and dendritic cells. Dendritic cells are a heterogeneous group of nonphagocytic antigen-presenting immune cells. Immunohistochemical markers help to distinguish among various primary histiocytic disorders. Characteristic radiologic findings in the appropriate clinical context may obviate biopsy to establish a correct diagnosis. However, in the absence of these findings, integration of clinical, pathologic, and radiologic features is required to establish a diagnosis.

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“…Among the various visceral manifestations of ECD, neurological and cardiac involvement has been described as indicators of poor prognosis [1,2]. Although radiological features of pleuropulmonary, mediastinal and cardiovascular involvement have been described previously [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16], the precise frequencies of their associations in patients remain unknown. The aim of this study was to evaluate the association among mediastinal, cardiac, pleural and pulmonary infiltration on computed tomography (CT) in a large series of 40 patients with biopsy-proven ECD.…”

Section: Introductionmentioning

confidence: 99%