Unusual variants of non-Langerhans cell histiocytoses

Caputo, Ruggero; Marzano, Angelo Valerio; Passoni, Emanuela; Berti, Emilio

doi:10.1016/j.jaad.2007.03.014

Cited by 60 publications

(60 citation statements)

References 77 publications

(167 reference statements)

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“…As mentioned, ECD shares clinical features with LCH (Table 2) but bears little clinical similarity to other non-LCH histiocytoses, with the exception of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). 24,25 Although infiltration of nearly every organ system has been reported in ECD, [26][27][28][29][30][31] the tissues most commonly affected include the skeleton, retroperitoneum, and orbit in addition to the cardiovascular, pulmonary, neurologic, and endocrine systems ( Figure 3). Below is a discussion of the organ systems affected by ECD with a consideration of the differential diagnosis for each regional presentation.…”

Section: 23mentioning

confidence: 99%

“…Intra-axially, ECD manifests more commonly in the dentate nuclei of the cerebellum or in the pons, causing progressive cerebellar symptoms such as ataxia and dysarthria as well as brainstem symptoms ( Figure 2E). 22,25,46 These abnormalities tend to be gadolinium-enhancing and may be mistaken for primary or metastatic tumors, demyelinating disease, inflammatory processes, or leukodystrophies. 28,29 Similar intracranial lesions have been reported in JXG.…”

Section: Consensus Guidelines For Erdheim-chester Disease 485mentioning

confidence: 99%

“…It is impossible to distinguish between ECD and adult JXG on the basis of skin lesions alone, but unlike ECD, JXG is less commonly a multi-system disease. 25 Proposed categories of disease. It has been reported that patients with CNS disease suffer adverse outcomes with interferon-based treatment compared with patients with other disease sites, supporting the notion that sites of involvement influence prognosis.…”

Section: Consensus Guidelines For Erdheim-chester Disease 485mentioning

confidence: 99%

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Consensus guidelines for the diagnosis and clinical management of Erdheim-Chester disease

Diamond

Dagna

Hyman

et al. 2014

Blood

513

695

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Erdheim-Chester disease (ECD) is a rare, non-Langerhans histiocytosis. Recent findings suggest that ECD is a clonal disorder, marked by recurrent BRAFV600E mutations in >50% of patients, in which chronic uncontrolled inflammation is an important mediator of disease pathogenesis. Although ∼500 to 550 cases have been described in the literature to date, increased physician awareness has driven a dramatic increase in ECD diagnoses over the last decade. ECD frequently involves multiple organ systems and has historically lacked effective therapies. Given the protean clinical manifestations and the lack of a consensus-derived approach for the management of ECD, we provide here the first multidisciplinary consensus guidelines for the clinical management of ECD. These recommendations were outlined at the First International Medical Symposium for ECD, comprised of a comprehensive group of international academicians with expertise in the pathophysiology and therapy of ECD. Detailed recommendations on the initial clinical, laboratory, and radiographic assessment of ECD patients are presented in addition to treatment recommendations based on critical appraisal of the literature and clinical experience. These formalized consensus descriptions will hopefully facilitate ongoing and future research efforts in this disorder. (Blood. 2014;124(4):483-492)

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Section: 23mentioning

confidence: 99%

Section: Consensus Guidelines For Erdheim-chester Disease 485mentioning

confidence: 99%

Section: Consensus Guidelines For Erdheim-chester Disease 485mentioning

confidence: 99%

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Consensus guidelines for the diagnosis and clinical management of Erdheim-Chester disease

Diamond

Dagna

Hyman

et al. 2014

Blood

513

695

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“…1,3,5,8,11,14,[16][17][18][19][20]23,[27][28][29][30][31][32] Erdheim-Chester disease is a multisystemic disease and can involve almost any organ system. Patients with ECD frequently have CNS involvement as an extraskeletal manifestation.…”

Section: Radiological Findingsmentioning

confidence: 99%

Erdheim-Chester disease mimicking an intracranial trigeminal schwannoma: case report

Alimohamadi

Hartmann

Paternó

et al. 2015

PED

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Erdheim-Chester disease (ECD) is non-Langerhans histiocytosis that can affect multiple organ systems. It usually affects middle-aged patients, and only a few reports of ECD in children appear in the literature. Central nervous system involvement is a common feature that usually occurs as infiltration of the hypothalamus-pituitary axis, cerebellum, and/or brainstem. Meningeal involvement occurs less commonly. In this article, the authors discuss a rare pediatric case of ECD presenting as an infiltrative mass of the trigeminal nerve and resembling the clinical and imaging features of a trigeminal schwannoma.

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“…2,3 Lesions tend to be diffuse and involve the face, typically with sparing of the mucous membranes, although some studies have noted involvement of oral and conjunctival mucosae. [3][4][5] Most patients with PNH have no systemic involvement, but there have been a few reported cases of associated malignancies. 5,6 The clinical course is characterized by the progressive appearance of new lesions.…”

Section: Discussionmentioning

confidence: 99%

Reddish-Brown Nodules and Papules in an Elderly Man

et al. 2013

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A man in his 70s with a 4-year history of stage IV chronic lymphocytic leukemia (CLL) presented with a 12-month history of an asymptomatic skin eruption. His CLL had been in remission for 2 years after treatment with fludarabine and rituximab before he relapsed and underwent 6 cycles of RCHOP treatment (rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone). During his CLL relapse, he developed cutaneous nodules on his face that then spread diffusely. The patient denied fevers, arthralgias, bone pain, polydipsia, or polyuria. Physical examination revealed multiple 0.5-to 1.5-cm reddish-brown firm papules and nodules on his cheeks, torso, buttocks, and extensor surfaces of his extremities (Figure, A), sparing his eyelids, palms, soles, and mucous membranes. Triglyceride levels were mildly elevated at 211 mg/dL; otherwise the patient's lipid levels were normal (to convert triglycerides to millimoles per liter, multiply by 0.0113). A biopsy of a representative papule on the thigh was performed, and a routine hematoxylin-eosinstained section (Figure, B) and CD68 immunohistochemically stained section (Figure, C) are shown. Findings of CD1a and S100 stainings were negative.

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Unusual variants of non-Langerhans cell histiocytoses

Cited by 60 publications

References 77 publications

Consensus guidelines for the diagnosis and clinical management of Erdheim-Chester disease

Consensus guidelines for the diagnosis and clinical management of Erdheim-Chester disease

Erdheim-Chester disease mimicking an intracranial trigeminal schwannoma: case report

Reddish-Brown Nodules and Papules in an Elderly Man

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