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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.
See: This record
Not genetically inherited (Orphanet)
 
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

Transfer factor for the treatment of necrobiotic xanthogranuloma.
Tang Z, Ruan J, Cai S
Dermatol Ther 2022 Dec;35(12):e15894. Epub 2022 Oct 18 doi: 10.1111/dth.15894. PMID: 36196500
Adult Xanthogranulomatous Disease of the Orbit: Clinical Presentations, Evaluation, and Management.
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
Treatment of necrobiotic xanthogranuloma - a systematic review.
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

Recent clinical studies

Etiology

Cutaneous paraneoplastic syndromes.
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
Recurrent Bilateral Periorbital Swelling.
Hellman JB, Kim E, Lin LK
JAMA Ophthalmol 2018 Dec 1;136(12):1406-1407. doi: 10.1001/jamaophthalmol.2018.1856. PMID: 30242331
Granulomatous diseases: Kids are not just little people.
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
Fifty shades of yellow: a review of the xanthodermatoses.
Frew JW, Murrell DF, Haber RM
Int J Dermatol 2015 Oct;54(10):1109-23. Epub 2015 Jul 30 doi: 10.1111/ijd.12945. PMID: 26227781
The expanding spectrum of cutaneous borreliosis.
Eisendle K, Zelger B
G Ital Dermatol Venereol 2009 Apr;144(2):157-71. PMID: 19357623

Diagnosis

Necrobiotic Xanthogranuloma.
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
Disseminated Necrobiotic Xanthogranuloma.
Navarro-Triviño F, Ródenas-Herranz T, Linares-Gonzalez L, Ruiz-Villaverde R
Med Clin (Barc) 2021 Nov 26;157(10):505. Epub 2021 Mar 26 doi: 10.1016/j.medcli.2020.12.038. PMID: 33775398
Necrobiotic Xanthogranuloma.
Dellatorre G, Miqueloto JK
JAMA Dermatol 2020 Jun 1;156(6):696. doi: 10.1001/jamadermatol.2020.0897. PMID: 32374351
Necrobiotic xanthogranuloma.
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
Necrobiotic xanthogranuloma.
Spicknall KE, Mehregan DA
Int J Dermatol 2009 Jan;48(1):1-10. doi: 10.1111/j.1365-4632.2009.03912.x. PMID: 19126043

Therapy

Periocular Necrobiotic Xanthogranuloma Response to Cladribine.
Agrawal S, Pushker N, Modaboyina S, Das D
Ophthalmic Plast Reconstr Surg 2022 Mar-Apr 01;38(2):e64. doi: 10.1097/IOP.0000000000002037. PMID: 34314397
Necrobiotic Xanthogranuloma.
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
Necrobiotic Xanthogranuloma.
Dellatorre G, Miqueloto JK
JAMA Dermatol 2020 Jun 1;156(6):696. doi: 10.1001/jamadermatol.2020.0897. PMID: 32374351
Necrobiotic xanthogranuloma.
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
Necrobiotic xanthogranuloma.
Rose A, Robinson M, Kamino H, Latkowski JA
Dermatol Online J 2012 Dec 15;18(12):30. PMID: 23286820

Prognosis

Recurrent Bilateral Periorbital Swelling.
Hellman JB, Kim E, Lin LK
JAMA Ophthalmol 2018 Dec 1;136(12):1406-1407. doi: 10.1001/jamaophthalmol.2018.1856. PMID: 30242331
Granulomatous diseases: Kids are not just little people.
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
Necrobiotic xanthogranuloma: response to dapsone.
Wei YH, Cheng JJ, Wu YH, Liu CY, Hung CJ, Hsu JD, Hsiao YP
Dermatol Ther 2015 Jan-Feb;28(1):7-9. Epub 2014 Oct 14 doi: 10.1111/dth.12179. PMID: 25314033
Necrobiotic xanthogranuloma with paraproteinemia.
Barzilai A, Trau H, Shpiro D, Yorav S
Cutis 1996 May;57(5):320-2. PMID: 8726711
Necrobiotic xanthogranuloma with cardiac involvement.
Umbert I, Winkelmann RK
Br J Dermatol 1995 Sep;133(3):438-43. doi: 10.1111/j.1365-2133.1995.tb02674.x. PMID: 8547001

Clinical prediction guides

Non-infectious granulomatous dermatoses.
Schmitt A, Volz A
J Dtsch Dermatol Ges 2019 May;17(5):518-533. doi: 10.1111/ddg.13848. PMID: 31115996
High prevalence of BRAF V600E mutations in Erdheim-Chester disease but not in other non-Langerhans cell histiocytoses.
Haroche J, Charlotte F, Arnaud L, von Deimling A, Hélias-Rodzewicz Z, Hervier B, Cohen-Aubart F, Launay D, Lesot A, Mokhtari K, Canioni D, Galmiche L, Rose C, Schmalzing M, Croockewit S, Kambouchner M, Copin MC, Fraitag S, Sahm F, Brousse N, Amoura Z, Donadieu J, Emile JF
Blood 2012 Sep 27;120(13):2700-3. Epub 2012 Aug 9 doi: 10.1182/blood-2012-05-430140. PMID: 22879539
Necrobiotic xanthogranuloma with paraproteinemia; an atypical case.
Ito Y, Nishimura K, Yamanaka K, Hakamada A, Isoda K, Kurokawa I, Mizutani H
J Dtsch Dermatol Ges 2008 Jan;6(1):40-3. Epub 2007 Oct 18 doi: 10.1111/j.1610-0387.2007.06392.x. PMID: 17941882
Necrobiotic xanthogranuloma.
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
Necrobiotic xanthogranuloma associated with myeloma.
Martínez Fernández M, Rodríguez Prieto MA, Ruiz González I, Sánchez Sambucety P, Delgado Vicente S
J Eur Acad Dermatol Venereol 2004 May;18(3):328-31. doi: 10.1111/j.1468-3083.2004.00906.x. PMID: 15096146

Recent systematic reviews

Systemic therapy of necrobiotic xanthogranuloma: a systematic review.
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
A Multicenter Cross-Sectional Study and Systematic Review of Necrobiotic Xanthogranuloma With Proposed Diagnostic Criteria.
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
Treatment of necrobiotic xanthogranuloma - a systematic review.
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

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