U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Cutaneous Erdheim-Chester Disease

MedGen UID:
1709068
•Concept ID:
C5239291
•
Neoplastic Process

Definition

Erdheim-Chester disease that affects the skin. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCutaneous Erdheim-Chester Disease

Professional guidelines

PubMed

Treatment of Langerhans cell histiocytosis: role of BRAF/MAPK inhibition.
Abla O, Weitzman S
Hematology Am Soc Hematol Educ Program 2015;2015:565-70. doi: 10.1182/asheducation-2015.1.565. PMID: 26637773

Recent clinical studies

Therapy

Erdheim-Chester Disease: A Case Report of BRAF V600E-Negative, MAP2K1-Positive ECD Diagnosed by Blood Next-Generation Sequencing Assay and a Brief Literature Review.
Aggarwal A, Taychert M, Hasanin L, Doll D, Basuino MG, Hasanein H
Oncology (Williston Park) 2023 Jul 21;37(7):298-302. doi: 10.46883/2023.25921001. PMID: 37499251
Erdheim-Chester disease: a comprehensive review from the ophthalmologic perspective.
Kanakis M, Petrou P, Lourida G, Georgalas I
Surv Ophthalmol 2022 Mar-Apr;67(2):388-410. Epub 2021 May 31 doi: 10.1016/j.survophthal.2021.05.013. PMID: 34081930
Clinical presentation, imaging and response to interferon-alpha therapy in Erdheim-Chester disease: case-based review.
Iaremenko O, Petelytska L, Dyadyk O, Negria N, Fedkov D
Rheumatol Int 2020 Sep;40(9):1529-1536. Epub 2020 Jun 22 doi: 10.1007/s00296-020-04627-z. PMID: 32572610
The role of 18FDG, 18FDOPA PET/CT and 99mTc bone scintigraphy imaging in Erdheim-Chester disease.
García-Gómez FJ, Acevedo-Báñez I, Martínez-Castillo R, Tirado-Hospital JL, Cuenca-Cuenca JI, Pachón-Garrudo VM, Álvarez-Pérez RM, García-Jiménez R, Rivas-Infante E, García-Morillo JS, Borrego-Dorado I
Eur J Radiol 2015 Aug;84(8):1586-1592. Epub 2015 Apr 30 doi: 10.1016/j.ejrad.2015.04.022. PMID: 25975897
Anakinra-responsive lichen planus in a woman with Erdheim-Chester disease: a therapeutic enigma.
Cohen PR, Kurzrock R
Dermatol Online J 2014 Jan 15;20(1):21241. PMID: 24456945

Prognosis

Erdheim-Chester Disease: A Case Report of BRAF V600E-Negative, MAP2K1-Positive ECD Diagnosed by Blood Next-Generation Sequencing Assay and a Brief Literature Review.
Aggarwal A, Taychert M, Hasanin L, Doll D, Basuino MG, Hasanein H
Oncology (Williston Park) 2023 Jul 21;37(7):298-302. doi: 10.46883/2023.25921001. PMID: 37499251
MAP2K1-driven mixed Langerhans cell histiocytosis, Rosai-Dorfman-Destombes disease and Erdheim-Chester disease, clonally related to acute myeloid leukemia.
Bonometti A, Ferrario G, Parafioriti A, Giardino D, Simonetti F, Ginori A, Passoni E, Berti E; for AIRI, Associazione Italiana Ricerca Istiocitosi ONLUS
J Cutan Pathol 2021 May;48(5):637-643. Epub 2020 Nov 24 doi: 10.1111/cup.13918. PMID: 33188581
The role of 18FDG, 18FDOPA PET/CT and 99mTc bone scintigraphy imaging in Erdheim-Chester disease.
García-Gómez FJ, Acevedo-Báñez I, Martínez-Castillo R, Tirado-Hospital JL, Cuenca-Cuenca JI, Pachón-Garrudo VM, Álvarez-Pérez RM, García-Jiménez R, Rivas-Infante E, García-Morillo JS, Borrego-Dorado I
Eur J Radiol 2015 Aug;84(8):1586-1592. Epub 2015 Apr 30 doi: 10.1016/j.ejrad.2015.04.022. PMID: 25975897
Multiple cutaneous, oropharyngeal and pulmonary nodules.
Veraitch O, Bhargava K, Moriarty B, Calonje E, Mallipeddi R, Whittaker S
Br J Dermatol 2015 Aug;173(2):540-3. Epub 2015 May 18 doi: 10.1111/bjd.13708. PMID: 25639756
Erdheim-Chester disease presenting with cutaneous involvement: a case report and literature review.
Volpicelli ER, Doyle L, Annes JP, Murray MF, Jacobsen E, Murphy GF, Saavedra AP
J Cutan Pathol 2011 Mar;38(3):280-5. Epub 2010 Dec 12 doi: 10.1111/j.1600-0560.2010.01650.x. PMID: 21143617Free PMC Article

Clinical prediction guides

Selected Case From the Arkadi M. Rywlin International Pathology Slide Seminar: Involvement of Skin and Soft Tissue by Erdheim-Chester Disease.
Suster DI, Ronen S, Suster S
Adv Anat Pathol 2024 Jul 1;31(4):271-274. Epub 2024 Jan 5 doi: 10.1097/PAP.0000000000000429. PMID: 38180007
Cutaneous manifestations of Erdheim-Chester disease (ECD): Clinical, pathological, and molecular features in a monocentric series of 40 patients.
Chasset F, Barete S, Charlotte F, Cohen-Aubart F, Arnaud L, Le Pelletier F, Emile JF, Francès C, Amoura Z, Haroche J
J Am Acad Dermatol 2016 Mar;74(3):513-20. Epub 2016 Jan 16 doi: 10.1016/j.jaad.2015.11.007. PMID: 26785805
Cerebral and Cutaneous Involvements of Xanthoma Disseminatum Successfully Treated with an Interleukin-1 Receptor Antagonist: A Case Report and Minireview.
Campero M, Campero S, Guerrero J, Aouba A, Castro A
Dermatology 2016;232(2):171-6. Epub 2016 Jan 8 doi: 10.1159/000442522. PMID: 26741816
The role of 18FDG, 18FDOPA PET/CT and 99mTc bone scintigraphy imaging in Erdheim-Chester disease.
García-Gómez FJ, Acevedo-Báñez I, Martínez-Castillo R, Tirado-Hospital JL, Cuenca-Cuenca JI, Pachón-Garrudo VM, Álvarez-Pérez RM, García-Jiménez R, Rivas-Infante E, García-Morillo JS, Borrego-Dorado I
Eur J Radiol 2015 Aug;84(8):1586-1592. Epub 2015 Apr 30 doi: 10.1016/j.ejrad.2015.04.022. PMID: 25975897

Supplemental Content

Table of contents

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Related information

    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...