MCID: NCR009
MIFTS: 26

Necrobiotic Xanthogranuloma

Categories: Rare diseases, Bone diseases, Skin diseases

Aliases & Classifications for Necrobiotic Xanthogranuloma

MalaCards integrated aliases for Necrobiotic Xanthogranuloma:

Name: Necrobiotic Xanthogranuloma 53 59 73
Nxg 53

Characteristics:

Orphanet epidemiological data:

59
necrobiotic xanthogranuloma
Inheritance: Not applicable;

Classifications:



External Ids:

Orphanet 59 ORPHA158011
MESH via Orphanet 45 D058252
UMLS via Orphanet 74 C1275339
ICD10 via Orphanet 34 D76.3
UMLS 73 C1275339

Summaries for Necrobiotic Xanthogranuloma

NIH Rare Diseases : 53 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 : 76 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 27)
# Related Disease Score Top Affiliating Genes
1 myeloma, multiple 10.4
2 scleritis 10.4
3 posterior scleritis 10.2
4 hepatitis 10.2
5 leukemia 10.2
6 leukemia, b-cell, chronic 10.2
7 lymphoma 10.2
8 leukemia, chronic lymphocytic 2 10.2
9 leukemia, chronic lymphocytic 10.2
10 uveitis 10.1
11 macroglobulinemia 9.9
12 neutropenia 9.9
13 amyloidosis 9.9
14 paroxysmal nocturnal hemoglobinuria 9.9
15 lymphoplasmacytic lymphoma 9.9
16 mucositis 9.9
17 portal hypertension 9.9
18 choroiditis 9.9
19 wegener granulomatosis 9.9
20 cerebritis 9.9
21 autoimmune lymphoproliferative syndrome, type v 9.9
22 necrobiosis lipoidica 9.9
23 burn scar 9.9
24 pancreatitis 9.9
25 burns 9.9
26 hemoglobinuria 9.9
27 mediastinitis 9.9

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:

41
Skin, Eye, Bone, Liver, Monocytes, Skeletal Muscle

Publications for Necrobiotic Xanthogranuloma

Articles related to Necrobiotic Xanthogranuloma:

(show top 50) (show all 160)
# Title Authors Year
1
Necrobiotic xanthogranuloma: a 30-year single-center experience. ( 29568990 )
2018
2
Necrobiotic xanthogranuloma associated with chronic lymphocytic leukemia. ( 29485184 )
2018
3
Necrobiotic xanthogranuloma: a paraneoplastic skin lesion of haematological malignancies? ( 29619997 )
2018
4
Periorbital Necrobiotic Xanthogranuloma Successfully Treated with Intravenous Immunoglobulin. ( 29643785 )
2018
5
Necrobiotic Xanthogranuloma: A Cutaneous Manifestation of Monoclonal Gammopathy. ( 29615198 )
2018
6
Necrobiotic xanthogranuloma treated with lenalidomide. ( 29048134 )
2017
7
Necrobiotic xanthogranuloma scleritis in a case of granulomatosis with polyangiitis (Wegener's granulomatosis). ( 29042820 )
2017
8
Rituximab Monotherapy Is Effective in Treating Orbital Necrobiotic Xanthogranuloma. ( 28863117 )
2017
9
Management of Uveitis and Scleritis in Necrobiotic Xanthogranuloma. ( 28394239 )
2017
10
Periocular Necrobiotic Xanthogranuloma Successfully Treated with Intravenous Immunoglobulin. ( 28127621 )
2017
11
Non-cirrhotic portal hypertension in necrobiotic xanthogranuloma associated with monoclonal gammopathy. ( 28295661 )
2017
12
Necrobiotic xanthogranuloma associated with monoclonal gammopathy of unknown significance. ( 28736067 )
2017
13
Image Gallery: Necrobiotic xanthogranuloma in association with chronic myelomonocytic leukaemia. ( 28244100 )
2017
14
Leprosy clinically masquerading as necrobiotic xanthogranuloma: a histopathological surprise! ( 28401539 )
2017
15
Hepatobiliary and Pancreatic: Hepatic necrobiotic xanthogranuloma. ( 28948701 )
2017
16
Necrobiotic Xanthogranuloma Treated With Topical Nitrogen Mustard (Mechlorethamine). ( 26762214 )
2016
17
Posterior Scleritis and Necrobiotic Xanthogranuloma. ( 26717063 )
2016
18
A Case of Necrobiotic Xanthogranuloma Presenting as Bilateral Posterior Scleritis. ( 27775456 )
2016
19
A rare case of disseminated skin and mucosal necrobiotic xanthogranuloma and xanthoma. ( 26810089 )
2016
20
Treatment of necrobiotic xanthogranuloma - a systematic review. ( 27436448 )
2016
21
Distinctive Pattern of Adipophilin Expression in Necrobiotic Xanthogranuloma. ( 26981735 )
2016
22
Ophthalmological considerations in necrobiotic xanthogranuloma. ( 26936405 )
2016
23
Necrobiotic Xanthogranuloma in a Patient with Multiple Myeloma. ( 28101022 )
2016
24
Necrobiotic xanthogranuloma treated with a combination of oral methylprednisolone and cyclosporin. ( 27790743 )
2016
25
Necrobiotic xanthogranuloma associated with monoclonal gammopathy: successful treatment with lenalidomide and dexamethasone. ( 26814914 )
2016
26
Clinical Features and Treatment Outcomes of Patients With Necrobiotic Xanthogranuloma Associated With Monoclonal Gammopathies. ( 27238425 )
2016
27
Hypercalcemia in Necrobiotic Xanthogranuloma: First Reported Case and Insight Into Treatment. ( 27886405 )
2016
28
Necrobiotic xanthogranuloma with giant cell hepatitis, successfully treated with intravenous immunoglobulins. ( 25650024 )
2015
29
Necrobiotic xanthogranuloma with multiple myeloma. ( 25809408 )
2015
30
Successful multimodality treatment of recalcitrant necrobiotic xanthogranuloma using electron beam radiation and intravenous immunoglobulin. ( 26206141 )
2015
31
Physiopathology of necrobiotic xanthogranuloma with monoclonal gammopathy. ( 24428816 )
2014
32
Successful treatment of necrobiotic xanthogranuloma with total glucosides of paeony. ( 24774012 )
2014
33
Necrobiotic xanthogranuloma: response to dapsone. ( 25314033 )
2014
34
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
35
Necrobiotic xanthogranuloma without a monoclonal gammopathy. ( 25566570 )
2014
36
18F-FDG PET/CT and MRI of Necrobiotic Xanthogranuloma. ( 23877513 )
2014
37
Necrobiotic xanthogranuloma. ( 24659748 )
2014
38
Vulvar necrobiotic xanthogranuloma. ( 25454046 )
2014
39
Solitary necrobiotic xanthogranuloma of an upper extremity in association with multiple myeloma. ( 24809886 )
2014
40
A multisystem granulomatous disease: necrobiotic xanthogranuloma with hepatic involvement. ( 24794614 )
2014
41
Bilateral necrobiotic xanthogranuloma of the eyelids followed by a diagnosis of multiple myeloma 20 years later. ( 23247037 )
2013
42
Successful treatment of necrobiotic xanthogranuloma with intravenous immunoglobulin. ( 23830027 )
2013
43
Successful treatment of necrobiotic xanthogranuloma with intravenous immunoglobulin. ( 24067856 )
2013
44
Treatment of necrobiotic xanthogranuloma with 2-chlorodeoxyadenosine. ( 23745232 )
2013
45
Periorbital necrobiotic xanthogranuloma treated successfully with novel multiple myeloma therapy. ( 24518382 )
2013
46
Immunomodulatory drugs for the treatment of periorbital necrobiotic xanthogranuloma. ( 24518383 )
2013
47
Necrobiotic xanthogranuloma as an unusual cause of refractive chronic bilateral leg ulceration. ( 23222162 )
2012
48
Necrobiotic xanthogranuloma of the extremities with paraproteinemia and without periorbital involvement at presentation. ( 22439311 )
2012
49
Necrobiotic xanthogranuloma and chronic lymphocytic leukemia of the conjunctiva masquerading as scleritis and uveitis. ( 23271884 )
2012
50
Necrobiotic xanthogranuloma. ( 23286820 )
2012

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 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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