MCID: NCR009
MIFTS: 26

Necrobiotic Xanthogranuloma

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 54 60 17 74
Nxg 54

Characteristics:

Orphanet epidemiological data:

60
necrobiotic xanthogranuloma
Inheritance: Not applicable;

Classifications:



External Ids:

MESH via Orphanet 46 D058252
ICD10 via Orphanet 35 D76.3
UMLS via Orphanet 75 C1275339
Orphanet 60 ORPHA158011
UMLS 74 C1275339

Summaries for Necrobiotic Xanthogranuloma

NIH Rare Diseases : 54 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 scleritis. An important gene associated with Necrobiotic Xanthogranuloma is IFNA2 (Interferon Alpha 2). Affiliated tissues include skin, eye and bone.

Wikipedia : 77 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 all 28)
# Related Disease Score Top Affiliating Genes
1 myeloma, multiple 10.6
2 scleritis 10.5
3 posterior scleritis 10.4
4 leukemia, chronic lymphocytic 2 10.3
5 leukemia, chronic lymphocytic 10.3
6 leukemia 10.3
7 lymphoma 10.3
8 lymphocytic leukemia 10.3
9 leukemia, b-cell, chronic 10.3
10 hepatitis 10.2
11 waldenstrom macroglobulinemia 10.2
12 uveitis 10.2
13 pelger-huet anomaly 10.1
14 leprosy 3 10.1
15 sjogren syndrome 10.1
16 granulomatosis with polyangiitis 10.1
17 burn scar 10.1
18 neutropenia 10.1
19 paroxysmal nocturnal hemoglobinuria 10.1
20 systemic scleroderma 10.1
21 lymphoproliferative syndrome 10.1
22 portal hypertension 10.1
23 necrobiosis lipoidica 10.1
24 hemoglobinuria 10.1
25 macroglobulinemia 10.1
26 amyloidosis 10.1
27 hansen's disease 10.1
28 human t-cell leukemia virus type 1 10.1

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:

42
Skin, Eye, Bone, Skeletal Muscle, Monocytes, T Cells, B Cells

Publications for Necrobiotic Xanthogranuloma

Articles related to Necrobiotic Xanthogranuloma:

(show top 50) (show all 163)
# Title Authors Year
1
Intravenous immunoglobulin for treatment of necrobiotic xanthogranuloma. ( 30221815 )
2019
2
Necrobiotic xanthogranuloma (without paraproteinemia) in a patient with Pelger-Huët anomaly. ( 30588610 )
2019
3
Extrafacial necrobiotic xanthogranuloma and monoclonal gammopathy: two cases. ( 30883695 )
2019
4
Necrobiotic xanthogranuloma associated with chronic lymphocytic leukemia. ( 29485184 )
2018
5
Rituximab Monotherapy Is Effective in Treating Orbital Necrobiotic Xanthogranuloma. ( 28863117 )
2018
6
Necrobiotic xanthogranuloma treated with lenalidomide. ( 29048134 )
2018
7
Necrobiotic xanthogranuloma: a 30-year single-center experience. ( 29568990 )
2018
8
Necrobiotic Xanthogranuloma: A Cutaneous Manifestation of Monoclonal Gammopathy. ( 29615198 )
2018
9
Necrobiotic xanthogranuloma: a paraneoplastic skin lesion of haematological malignancies? ( 29619997 )
2018
10
Periorbital Necrobiotic Xanthogranuloma Successfully Treated with Intravenous Immunoglobulin. ( 29643785 )
2018
11
Multicenter Evaluation of the Xpert MRSA NxG Assay for Detection of Methicillin-Resistant Staphylococcus aureus in Nasal Swabs. ( 29118165 )
2018
12
Necrobiotic xanthogranuloma scleritis in a case of granulomatosis with polyangiitis (Wegener's granulomatosis). ( 29042820 )
2017
13
A rare case of disseminated skin and mucosal necrobiotic xanthogranuloma and xanthoma. ( 26810089 )
2017
14
Treatment of necrobiotic xanthogranuloma - a systematic review. ( 27436448 )
2017
15
Necrobiotic xanthogranuloma treated with a combination of oral methylprednisolone and cyclosporin. ( 27790743 )
2017
16
Hypercalcemia in Necrobiotic Xanthogranuloma: First Reported Case and Insight Into Treatment. ( 27886405 )
2017
17
Periocular Necrobiotic Xanthogranuloma Successfully Treated with Intravenous Immunoglobulin. ( 28127621 )
2017
18
Image Gallery: Necrobiotic xanthogranuloma in association with chronic myelomonocytic leukaemia. ( 28244100 )
2017
19
Non-cirrhotic portal hypertension in necrobiotic xanthogranuloma associated with monoclonal gammopathy. ( 28295661 )
2017
20
Management of Uveitis and Scleritis in Necrobiotic Xanthogranuloma. ( 28394239 )
2017
21
Leprosy clinically masquerading as necrobiotic xanthogranuloma: a histopathological surprise! ( 28401539 )
2017
22
Necrobiotic xanthogranuloma associated with monoclonal gammopathy of unknown significance. ( 28736067 )
2017
23
Hepatobiliary and Pancreatic: Hepatic necrobiotic xanthogranuloma. ( 28948701 )
2017
24
Successful multimodality treatment of recalcitrant necrobiotic xanthogranuloma using electron beam radiation and intravenous immunoglobulin. ( 26206141 )
2016
25
Posterior Scleritis and Necrobiotic Xanthogranuloma. ( 26717063 )
2016
26
Necrobiotic Xanthogranuloma Treated With Topical Nitrogen Mustard (Mechlorethamine). ( 26762214 )
2016
27
Necrobiotic xanthogranuloma associated with monoclonal gammopathy: successful treatment with lenalidomide and dexamethasone. ( 26814914 )
2016
28
Ophthalmological considerations in necrobiotic xanthogranuloma. ( 26936405 )
2016
29
Distinctive Pattern of Adipophilin Expression in Necrobiotic Xanthogranuloma. ( 26981735 )
2016
30
Clinical Features and Treatment Outcomes of Patients With Necrobiotic Xanthogranuloma Associated With Monoclonal Gammopathies. ( 27238425 )
2016
31
A Case of Necrobiotic Xanthogranuloma Presenting as Bilateral Posterior Scleritis. ( 27775456 )
2016
32
Necrobiotic Xanthogranuloma in a Patient with Multiple Myeloma. ( 28101022 )
2016
33
Necrobiotic xanthogranuloma: response to dapsone. ( 25314033 )
2015
34
Necrobiotic xanthogranuloma with giant cell hepatitis, successfully treated with intravenous immunoglobulins. ( 25650024 )
2015
35
Necrobiotic xanthogranuloma with multiple myeloma. ( 25809408 )
2015
36
Generalized Necrobiotic Xanthogranuloma in a Patient with Multiple Myelomaus Patient with Isolated Neutropenia and Diminished Expression of CD55 and CD59 Similar to Paroxysmal Nocturnal Hemoglobinuria. ( 25541670 )
2014
37
18F-FDG PET/CT and MRI of necrobiotic xanthogranuloma. ( 23877513 )
2014
38
Physiopathology of necrobiotic xanthogranuloma with monoclonal gammopathy. ( 24428816 )
2014
39
Necrobiotic xanthogranuloma. ( 24659748 )
2014
40
Successful treatment of necrobiotic xanthogranuloma with total glucosides of paeony. ( 24774012 )
2014
41
A multisystem granulomatous disease: necrobiotic xanthogranuloma with hepatic involvement. ( 24794614 )
2014
42
Solitary necrobiotic xanthogranuloma of an upper extremity in association with multiple myeloma. ( 24809886 )
2014
43
Vulvar necrobiotic xanthogranuloma. ( 25454046 )
2014
44
Necrobiotic xanthogranuloma without a monoclonal gammopathy. ( 25566570 )
2014
45
[Necrobiotic xanthogranuloma. Differential diagnosis, treatment and systemic involvement. Case report]. ( 24269389 )
2014
46
Bilateral necrobiotic xanthogranuloma of the eyelids followed by a diagnosis of multiple myeloma 20 years later. ( 23247037 )
2013
47
Treatment of necrobiotic xanthogranuloma with 2-chlorodeoxyadenosine. ( 23745232 )
2013
48
Successful treatment of necrobiotic xanthogranuloma with intravenous immunoglobulin. ( 24067856 )
2013
49
Periorbital necrobiotic xanthogranuloma treated successfully with novel multiple myeloma therapy. ( 24518382 )
2013
50
Immunomodulatory drugs for the treatment of periorbital necrobiotic xanthogranuloma. ( 24518383 )
2013

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 IFNA2 MSR1

Sources for Necrobiotic Xanthogranuloma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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