Erdheim–Chester Disease

Murray, Drew; Marshall, Martin; England, E.J.; Mander, Julian; Chakera, T. M. H.

doi:10.1053/crad.2001.0681

Cited by 63 publications

(42 citation statements)

References 9 publications

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“…6) [9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25][26]. In nessuno dei nostri pazienti è stato osservato esoftalmo, sebbene rappresenti una frequente presentazione neurologica di ECD, conseguente ad una infiltrazione del tessuto adiposo retroconale e della guaina del nervo ottico.…”

Section: A B Cunclassified

Erdheim-Chester disease: clinical and radiological findings

et al. 2009

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Section: A B Cunclassified

Erdheim-Chester disease: clinical and radiological findings

et al. 2009

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“…Distal femur and proximal tibia and fibula are the most frequently affected sites, whereas the disease usually spares the axial skeleton and flat bones. 57,58 In addition to the sclerotic changes, lytic lesions can be seen in up to 30% of cases. 59 Pathologic bone fracture, compression of the femoral neck, and the development of bone abscesses with cutaneous perforation have all been proposed as potential mechanisms involved in the manifestation of bone pain in ECD.…”

Section: Clinical Findingsmentioning

confidence: 99%

“…Although retroperitoneal and renal involvement occurs in one third of patients and may manifest clinically as abdominal pain, dysuria, and hydronephrosis, the majority of cases are asymptomatic. 57,58 Pulmonary involvement is uncommon, occurring in approximately 20% of reported patients, and is characterized by a progressive course commonly manifesting with dyspnea. 62 Radiographic changes consist of diffuse interstitial infiltrates and pleural and/or interlobular septal thickening.…”

Section: Clinical Findingsmentioning

confidence: 99%

Unusual variants of non-Langerhans cell histiocytoses

Caputo¹,

Marzano²,

Passoni³

et al. 2007

Journal of the American Academy of Dermatology

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Histiocytic syndromes represent a large, heterogeneous group of diseases resulting from proliferation of histiocytes. In addition to the classic variants, the subset of non-Langerhans cell histiocytoses comprises rare entities that have more recently been described. These last include both forms that affect only the skin or the skin and mucous membranes, and usually show a benign clinical behavior, and forms involving also internal organs, which may follow an aggressive course. The goal of this review is to outline the clinical, histologic, and ultrastructural features and the course, prognosis, and management of these unusual histiocytic syndromes.

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“…Typically, bone pain is the most frequent symptom, 2,5,15 with underlying cortical osteosclerosis of long bones and abnormally increased labeling in the long bones of the lower limbs on technetium-99 bone scans. 5,15,16 Half of patients however have extra-skeletal manifestations including exophthalmos, interstitial lung disease, diabetes insipidus, and xanthelasma. 3,5,17 Interferon-alpha is generally used as first line therapy for ECD, but given the variable response, treatment is individualized depending on extent and severity of organ involved.…”

Section: Discussionmentioning

confidence: 99%