NXG
MCID: NCR009
MIFTS: 26

Necrobiotic Xanthogranuloma (NXG)

Categories: Bone diseases, Eye diseases, Immune diseases, Rare diseases, Skin diseases

Aliases & Classifications for Necrobiotic Xanthogranuloma

MalaCards integrated aliases for Necrobiotic Xanthogranuloma:

Name: Necrobiotic Xanthogranuloma 20 58 17 70
Nxg 20

Characteristics:

Orphanet epidemiological data:

58
necrobiotic xanthogranuloma
Inheritance: Not applicable;

Classifications:

Orphanet: 58  
Rare systemic and rhumatological diseases
Rare skin diseases


External Ids:

MESH via Orphanet 45 D058252
ICD10 via Orphanet 33 D76.3
UMLS via Orphanet 71 C1275339
Orphanet 58 ORPHA158011
UMLS 70 C1275339

Summaries for Necrobiotic Xanthogranuloma

GARD : 20 Necrobiotic xanthogranuloma (NXG) is a rare, chronic form of non-Langerhans histiocytosis usually found in older adults. Xanthogranulomas are lesions made of immune cells known as a histiocytes. The term necrobiotic refers to the buildup of broken down collagen fibers that can be seen under a microscope. The typical lesion is a yellow, thickened, or raised lesion (plaque) located around the eyes. In most cases the lesions are associated with conditions in which abnormal proteins are found in the blood (monoclonal gammopathies). NXG is also sometimes associated with blood cancers or lymphoproliferative disorders. As these blood disorders may arise years after the first NXG lesions appear, lifelong follow-up may be recommended. Approximately 50% of people with NXG have problems with their eyes, such as burning or itching, blurred vision, double vision, bulging of the eyeball, drooping of the eyelid, and restricted eye movement. In most cases, other parts of the body, such as the trunk, legs, face, and arms, are also involved. Internal organs may sometimes be affected. Treatment of NXG can be challenging, but is usually necessary to minimize the risk of skin ulcers and scarring. First-line therapy may include drugs used to treat cancer (such as chlorambucil and melphalan ). Additional treatment options include corticosteroids, intravenous immunoglobulin, lenalidomide, interferon, radiation therapy, and surgery. In some cases, the symptoms of NXG may return after treatment. If NXG is associated with a blood cancer or a lymphoproliferative disorder, treatment focuses on the blood disorder.

MalaCards based summary : Necrobiotic Xanthogranuloma, also known as nxg, is related to myeloma, multiple and histiocytosis. An important gene associated with Necrobiotic Xanthogranuloma is IFNA2 (Interferon Alpha 2). Affiliated tissues include eye, skin and skeletal muscle.

Wikipedia : 73 Necrobiotic xanthogranuloma (also known as "necrobiotic xanthogranuloma with paraproteinemia") is a... more...

Related Diseases for Necrobiotic Xanthogranuloma

Diseases related to Necrobiotic Xanthogranuloma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 75)
# Related Disease Score Top Affiliating Genes
1 myeloma, multiple 10.7
2 histiocytosis 10.6
3 scleritis 10.6
4 necrobiosis lipoidica 10.6
5 leukemia, chronic lymphocytic 10.4
6 langerhans cell histiocytosis 10.4
7 non-langerhans-cell histiocytosis 10.4
8 plasma cell neoplasm 10.4
9 posterior scleritis 10.4
10 granuloma annulare 10.4
11 cryoglobulinemia 10.3
12 skin disease 10.3
13 keratitis, hereditary 10.3
14 xanthomatosis 10.3
15 lymphoproliferative syndrome 10.3
16 uveitis 10.3
17 portal hypertension 10.2
18 neutropenia 10.2
19 panniculitis 10.2
20 conjunctivitis 10.2
21 macroglobulinemia 10.2
22 amyloidosis 10.2
23 lichen sclerosus et atrophicus 10.0
24 pelger-huet anomaly 10.0
25 sarcoidosis 1 10.0
26 varicose veins 10.0
27 lung cancer 10.0
28 lymphoma, hodgkin, classic 10.0
29 leprosy 3 10.0
30 yemenite deaf-blind hypopigmentation syndrome 10.0
31 granulomatosis with polyangiitis 10.0
32 proteinuria, chronic benign 10.0
33 lymphoplasmacytic lymphoma 10.0
34 c1 inhibitor deficiency 10.0
35 cogan syndrome 10.0
36 ptosis 10.0
37 paroxysmal nocturnal hemoglobinuria 10.0
38 erythema elevatum diutinum 10.0
39 polymyositis 10.0
40 esophageal varix 10.0
41 pulmonary edema 10.0
42 cardiac tamponade 10.0
43 multicentric reticulohistiocytosis 10.0
44 nephrotic syndrome 10.0
45 mononeuropathy 10.0
46 panuveitis 10.0
47 leukemia 10.0
48 pancytopenia 10.0
49 hepatitis a 10.0
50 skin sarcoidosis 10.0

Graphical network of the top 20 diseases related to Necrobiotic Xanthogranuloma:



Diseases related to Necrobiotic Xanthogranuloma

Symptoms & Phenotypes for Necrobiotic Xanthogranuloma

Drugs & Therapeutics for Necrobiotic Xanthogranuloma

Search Clinical Trials , NIH Clinical Center for Necrobiotic Xanthogranuloma

Genetic Tests for Necrobiotic Xanthogranuloma

Anatomical Context for Necrobiotic Xanthogranuloma

MalaCards organs/tissues related to Necrobiotic Xanthogranuloma:

40
Eye, Skin, Skeletal Muscle, Monocytes, Pituitary, Lung

Publications for Necrobiotic Xanthogranuloma

Articles related to Necrobiotic Xanthogranuloma:

(show top 50) (show all 252)
# Title Authors PMID Year
1
Histiocytosis and Neoplasms of Macrophage-Dendritic Cell Lineages: Multimodality Imaging with Emphasis on PET/CT. 61
33606566 2021
2
Disseminated Necrobiotic Xanthogranuloma. 61
33775398 2021
3
Necrobiotic xanthogranuloma in IgG-κ multiple myeloma. 61
33529369 2021
4
Application of 18F-FDG PET/CT in necrobiotic xanthogranuloma involving multiple organs. 61
32409893 2021
5
Necrobiotic xanthogranuloma and polymyositis in a patient with squamous cell lung cancer: a new paraneoplastic entity? 61
33237031 2020
6
Necrobiotic Xanthogranuloma on 18F-FDG PET/CT. 61
32969908 2020
7
Successful treatment of highly refractory necrobiotic xanthogranuloma with peginterferon alfa-2a. 61
33222242 2020
8
Adult-Onset Asthma with Periocular Xanthogranuloma (AAPOX), a Variant of Periorbital Xanthogranulomatous Disease: An Uncommon Entity. 61
33235848 2020
9
Efficacy of Topical Use Crisaborole 2% Ointment for Treatment of Necrobiotic Xanthogranuloma Associated With Multiple Myeloma. 61
32389673 2020
10
Periocular Necrobiotic Xanthogranuloma-A Case of Unseen Eyes. 61
32789521 2020
11
Visual Dermatology: Necrobiotic Xanthogranuloma. 61
32726147 2020
12
Necrobiotic Xanthogranuloma. 61
32374351 2020
13
Necrobiotic xanthogranuloma developing in a patient with diffuse normolipemic plane xanthoma: association of two monoclonal gammopathy-related disorders. 61
31736053 2020
14
Clinical-Radiological Patterns and Histopathological Outcomes in Non-Thyroid Extraocular Muscle Enlargement: Retrospective Case Series and Current Concepts. 61
31789785 2020
15
A Multicenter Cross-Sectional Study and Systematic Review of Necrobiotic Xanthogranuloma With Proposed Diagnostic Criteria. 61
31940000 2020
16
An Exuberant Case of Necrobiotic Xanthogranuloma. 61
32055516 2020
17
[Periocular Necrobiotic Xanthogranuloma with Mono- and Biclonal Gammopathy]. 61
31968365 2020
18
Adult Onset Xantogranulomatous Disease Associated with Vitiligo. 61
31807456 2019
19
[Paraneoplastic skin diseases]. 61
31273399 2019
20
Cutaneous paraneoplastic syndromes. 61
30736994 2019
21
Necrobiotic xanthogranuloma associated with smoldering multiple myeloma: satisfactory response to cyclophosphamide, dexamethasone, and thalidomide. 61
31365665 2019
22
Primary Necrobiotic Xanthogranulomatous Sialadenitis with Submandibular Gland Localization without Skin Involvement. 61
30646671 2019
23
Extrafacial necrobiotic xanthogranuloma and monoclonal gammopathy: two cases. 61
30883695 2019
24
Necrobiotic xanthogranuloma associated with smoldering multiple myeloma: satisfactory response to cyclophosphamide, dexamethasone, and thalidomide. 61
33272543 2019
25
Necrobiotic xanthogranuloma (without paraproteinemia) in a patient with Pelger-Huët anomaly. 61
30588610 2019
26
[Necrobiotic xanthogranuloma misdiagnosed as a planocellular carcinoma]. 61
30950371 2019
27
Necrobiotic Xanthogranuloma as the Presenting Manifestation of Smouldering Myeloma. 61
31304718 2019
28
An unusual orbital tumor in an adult: Granuloma annulare. 61
30505978 2019
29
Intravenous immunoglobulin for treatment of necrobiotic xanthogranuloma. 61
30221815 2019
30
Necrobiotic Xanthogranuloma: A Cutaneous Manifestation of Monoclonal Gammopathy. 61
29615198 2018
31
Necrobiotic xanthogranuloma: a 30-year single-center experience. 61
29568990 2018
32
Necrobiotic xanthogranuloma associated with chronic lymphocytic leukemia. 61
29485184 2018
33
Necrobiotic xanthogranuloma: a paraneoplastic skin lesion of haematological malignancies? 61
29619997 2018
34
Necrobiotic xanthogranuloma treated with lenalidomide. 61
29048134 2018
35
Rituximab Monotherapy Is Effective in Treating Orbital Necrobiotic Xanthogranuloma. 61
28863117 2018
36
Periorbital Necrobiotic Xanthogranuloma Successfully Treated with Intravenous Immunoglobulin. 61
29643785 2018
37
Necrobiotic xanthogranuloma associated with monoclonal gammopathy of unknown significance. 61
28736067 2017
38
Necrobiotic xanthogranuloma treated with a combination of oral methylprednisolone and cyclosporin. 61
27790743 2017
39
Hepatobiliary and Pancreatic: Hepatic necrobiotic xanthogranuloma. 61
28948701 2017
40
The clinical spectrum of xanthomatous lesions of the eyelids. 61
28500693 2017
41
Leprosy clinically masquerading as necrobiotic xanthogranuloma: a histopathological surprise! 61
28401539 2017
42
Non-cirrhotic portal hypertension in necrobiotic xanthogranuloma associated with monoclonal gammopathy. 61
28295661 2017
43
[Clinical and histological spectrum of palisaded granulomatous dermatitides : Granuloma annulare, necrobiosis lipoidica, rheumatoid nodules, and necrobiotic xanthogranuloma]. 61
28567509 2017
44
Xanthoma-like Skin Changes in an Elderly Woman with a Normal Lipid Profile. 61
28871936 2017
45
Adult Xanthogranulomatous Disease of the Orbit: Clinical Presentations, Evaluation, and Management. 61
28262109 2017
46
Periocular Necrobiotic Xanthogranuloma Successfully Treated with Intravenous Immunoglobulin. 61
28127621 2017
47
Management of Uveitis and Scleritis in Necrobiotic Xanthogranuloma. 61
28394239 2017
48
Hypercalcemia in Necrobiotic Xanthogranuloma: First Reported Case and Insight Into Treatment. 61
27886405 2017
49
Image Gallery: Necrobiotic xanthogranuloma in association with chronic myelomonocytic leukaemia. 61
28244100 2017
50
Treatment of necrobiotic xanthogranuloma - a systematic review. 61
27436448 2017

Variations for Necrobiotic Xanthogranuloma

Expression for Necrobiotic Xanthogranuloma

Search GEO for disease gene expression data for Necrobiotic Xanthogranuloma.

Pathways for Necrobiotic Xanthogranuloma

GO Terms for Necrobiotic Xanthogranuloma

Biological processes related to Necrobiotic Xanthogranuloma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 negative regulation of gene expression GO:0010629 8.62 MSR1 IFNA2

Sources for Necrobiotic Xanthogranuloma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 20-May-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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