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Necrobiotic xanthogranuloma

MedGen UID:
266158
Concept ID:
C1275339
Disease or Syndrome
Synonyms: Necrobiotic Xanthogranuloma; Necrobiotic Xanthogranulomas; Xanthogranuloma, Necrobiotic; Xanthogranulomas, Necrobiotic
SNOMED CT: Necrobiotic xanthogranuloma (404164003)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0015537
Orphanet: ORPHA158011

Definition

Necrobiotic xanthogranuloma is a rare, chronic and progressive, non-Langerhans cell histiocytosis disease typically characterized by multiple, indurated, asymptomatic to pruritic, yellow-orange plaques or nodules that tend to ulcerate and are usually located in the periorbital area, trunk and/or extremities. Strong association with paraproteinemia and/or malignant lymphoproliferative disease has been reported. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVNecrobiotic xanthogranuloma
Follow this link to review classifications for Necrobiotic xanthogranuloma in Orphanet.

Professional guidelines

PubMed

Ortiz Salvador JM, Subiabre Ferrer D, Pérez Ferriols A
Actas Dermosifiliogr 2017 Jun;108(5):400-406. Epub 2017 Mar 3 doi: 10.1016/j.ad.2016.12.005. PMID: 28262109
Miguel D, Lukacs J, Illing T, Elsner P
J Eur Acad Dermatol Venereol 2017 Feb;31(2):221-235. Epub 2016 Jul 19 doi: 10.1111/jdv.13786. PMID: 27436448
Abdul-Hay M
Clin Adv Hematol Oncol 2013 Oct;11(10):680-1. PMID: 24518383

Recent clinical studies

Etiology

Wick MR, Patterson JW
Semin Diagn Pathol 2019 Jul;36(4):211-228. Epub 2019 Jan 31 doi: 10.1053/j.semdp.2019.01.001. PMID: 30736994
Baykal C, Polat Ekinci A, Yazganoglu KD, Buyukbabani N
Int J Dermatol 2017 Oct;56(10):981-992. Epub 2017 May 13 doi: 10.1111/ijd.13637. PMID: 28500693
Ortiz Salvador JM, Subiabre Ferrer D, Pérez Ferriols A
Actas Dermosifiliogr 2017 Jun;108(5):400-406. Epub 2017 Mar 3 doi: 10.1016/j.ad.2016.12.005. PMID: 28262109
Guo J, Wang J
Arch Pathol Lab Med 2009 Dec;133(12):1994-7. doi: 10.5858/133.12.1994. PMID: 19961259
Eisendle K, Zelger B
G Ital Dermatol Venereol 2009 Apr;144(2):157-71. PMID: 19357623

Diagnosis

Portuguese AJ, Long TH, Linenberger M
Mayo Clin Proc 2021 Sep;96(9):2432-2434. doi: 10.1016/j.mayocp.2021.05.012. PMID: 34481598
Dellatorre G, Miqueloto JK
JAMA Dermatol 2020 Jun 1;156(6):696. doi: 10.1001/jamadermatol.2020.0897. PMID: 32374351
Spicknall KE, Mehregan DA
Int J Dermatol 2009 Jan;48(1):1-10. doi: 10.1111/j.1365-4632.2009.03912.x. PMID: 19126043
Fernández-Herrera J, Pedraz J
Semin Cutan Med Surg 2007 Jun;26(2):108-13. doi: 10.1016/j.sder.2007.02.008. PMID: 17544963
Mehregan DA, Winkelmann RK
Arch Dermatol 1992 Jan;128(1):94-100. PMID: 1739294

Therapy

Portuguese AJ, Long TH, Linenberger M
Mayo Clin Proc 2021 Sep;96(9):2432-2434. doi: 10.1016/j.mayocp.2021.05.012. PMID: 34481598
Dellatorre G, Miqueloto JK
JAMA Dermatol 2020 Jun 1;156(6):696. doi: 10.1001/jamadermatol.2020.0897. PMID: 32374351
Miguel D, Lukacs J, Illing T, Elsner P
J Eur Acad Dermatol Venereol 2017 Feb;31(2):221-235. Epub 2016 Jul 19 doi: 10.1111/jdv.13786. PMID: 27436448
Jaén-Águila F, Vargas-Hitos JA, Jiménez-Alonso J
QJM 2014 Oct;107(10):859. Epub 2014 Mar 22 doi: 10.1093/qjmed/hcu066. PMID: 24659748
Rose A, Robinson M, Kamino H, Latkowski JA
Dermatol Online J 2012 Dec 15;18(12):30. PMID: 23286820

Prognosis

Lakdawala N, Ferenczi K, Grant-Kels JM
Clin Dermatol 2017 Nov-Dec;35(6):555-565. Epub 2017 Aug 4 doi: 10.1016/j.clindermatol.2017.08.006. PMID: 29191347
Baykal C, Polat Ekinci A, Yazganoglu KD, Buyukbabani N
Int J Dermatol 2017 Oct;56(10):981-992. Epub 2017 May 13 doi: 10.1111/ijd.13637. PMID: 28500693
Miguel D, Lukacs J, Illing T, Elsner P
J Eur Acad Dermatol Venereol 2017 Feb;31(2):221-235. Epub 2016 Jul 19 doi: 10.1111/jdv.13786. PMID: 27436448
Fernández-Herrera J, Pedraz J
Semin Cutan Med Surg 2007 Jun;26(2):108-13. doi: 10.1016/j.sder.2007.02.008. PMID: 17544963
Mehregan DA, Winkelmann RK
Arch Dermatol 1992 Jan;128(1):94-100. PMID: 1739294

Clinical prediction guides

Popoola D, Housley SB, Jacoby WT, Lim J, Cappuzzo JM, Levy EI
BMJ Case Rep 2023 Aug 30;16(8) doi: 10.1136/bcr-2022-254122. PMID: 37648279Free PMC Article
Nelson CA, Zhong CS, Hashemi DA, Ashchyan HJ, Brown-Joel Z, Noe MH, Imadojemu S, Micheletti RG, Vleugels RA, Wanat KA, Rosenbach M, Mostaghimi A
JAMA Dermatol 2020 Mar 1;156(3):270-279. doi: 10.1001/jamadermatol.2019.4221. PMID: 31940000Free PMC Article
Schmitt A, Volz A
J Dtsch Dermatol Ges 2019 May;17(5):518-533. doi: 10.1111/ddg.13848. PMID: 31115996
Zelger B
G Ital Dermatol Venereol 2013 Aug;148(4):351-70. PMID: 23900158
Fernández-Herrera J, Pedraz J
Semin Cutan Med Surg 2007 Jun;26(2):108-13. doi: 10.1016/j.sder.2007.02.008. PMID: 17544963

Recent systematic reviews

Steinhelfer L, Kühnel T, Jägle H, Mayer S, Karrer S, Haubner F, Schreml S
Orphanet J Rare Dis 2022 Mar 24;17(1):132. doi: 10.1186/s13023-022-02291-z. PMID: 35331271Free PMC Article
Nelson CA, Zhong CS, Hashemi DA, Ashchyan HJ, Brown-Joel Z, Noe MH, Imadojemu S, Micheletti RG, Vleugels RA, Wanat KA, Rosenbach M, Mostaghimi A
JAMA Dermatol 2020 Mar 1;156(3):270-279. doi: 10.1001/jamadermatol.2019.4221. PMID: 31940000Free PMC Article
Miguel D, Lukacs J, Illing T, Elsner P
J Eur Acad Dermatol Venereol 2017 Feb;31(2):221-235. Epub 2016 Jul 19 doi: 10.1111/jdv.13786. PMID: 27436448

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

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    • Bookshelf
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