MCID: LYM052
MIFTS: 48

Lymphomatoid Papulosis malady

Categories: Rare diseases, Skin diseases, Blood diseases, Cancer diseases, Immune diseases

Aliases & Classifications for Lymphomatoid Papulosis

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Sources:
45NIH Rare Diseases, 47Novoseek, 51Orphanet, 65UMLS, 28ICD10 via Orphanet, 37MESH via Orphanet, 66UMLS via Orphanet, 27ICD10
See all MalaCards sources

Aliases & Descriptions for Lymphomatoid Papulosis:

Name: Lymphomatoid Papulosis 45 47 51 65
Lyp 45 51
 
X-Linked Lymphoproliferative Disorder 65

Characteristics:

Orphanet epidemiological data:

51
lymphomatoid papulosis:
Age of onset: All ages; Age of death: normal life expectancy

Classifications:



External Ids:

Orphanet51 98842
ICD10 via Orphanet28 C86.6
MESH via Orphanet37 D017731
UMLS via Orphanet66 C0206182
ICD1027 L41.2
UMLS65 C0206182, C0549463

Summaries for Lymphomatoid Papulosis

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NIH Rare Diseases:45 Lymphomatoid papulosis is a skin disorder that is characterized by crops of self healing skin lesions that look cancerous under the microscope but are actually benign (non-cancerous). lesions contain unusual cells that are similar to those found in some lymphomas (cancers of the lymphatic system). last updated: 6/19/2012

MalaCards based summary: Lymphomatoid Papulosis, also known as lyp, is related to pityriasis lichenoides chronica and alk-negative anaplastic large cell lymphoma, and has symptoms including hepatomegaly An important gene associated with Lymphomatoid Papulosis is NPM1 (Nucleophosmin (Nucleolar Phosphoprotein B23, Numatrin)), and among its related pathways are IL12-mediated signaling events and African trypanosomiasis. Affiliated tissues include skin, t cells and breast, and related mouse phenotypes are tumorigenesis and respiratory system.

Wikipedia:68 Lymphomatoid papulosis (LyP) is a rare skin disorder. The overall prevalence rate of lymphomatoid... more...

Related Diseases for Lymphomatoid Papulosis

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Diseases related to Lymphomatoid Papulosis via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50)    (show all 86)
idRelated DiseaseScoreTop Affiliating Genes
1pityriasis lichenoides chronica30.9CD7, TNFRSF8
2alk-negative anaplastic large cell lymphoma30.9ALK, NPM1
3lymphoma10.9
4pityriasis rotunda10.7CD7, TNFRSF8
5nervous system disease10.7ALK, TNFRSF8
6nodular nonsuppurative panniculitis10.6CD7, TNFRSF8
7anaplastic large cell lymphoma10.6
8mycosis fungoides10.6
9tertiary neurosyphilis10.6GZMB, TIA1
10necrotizing gastritis10.6GZMB, TIA1
11ectopic thymus10.5GZMB, TIA1
12dilated cardiomyopathy10.5ALK, IRF4, TNFRSF8
13pityriasis lichenoides10.5
14pediatric fibrosarcoma10.5PDGFRA, TNFRSF8
15anterior horn cell disease10.4ALK, PDGFRA
16cutaneous t cell lymphoma10.4
17small intestine cancer10.4GZMB, PDGFRA, TIA1
18bard1-related susceptibility to breast cancer10.4CXCR3, TRG
19cell type benign neoplasm10.4ALK, CD7, NPM1, TNFRSF8
20primary cutaneous anaplastic large cell lymphoma10.4
21pol iii-related leukodystrophies10.4ALK, CXCR3, TIA1
22congenital sucrase-isomaltase deficiency without starch intolerance10.3ALK, CCR4
23pityriasis lichenoides et varioliformis acuta10.3
24swayback10.3HBB, TNFRSF8
25ductal carcinoma in situ10.3ALK, GZMB, NPM1, TIA1
26lymphopenia10.3
27histiocytosis10.3
28wild type abeta2m amyloidosis10.2FAS, TYK2
29leukemia10.2
30hypereosinophilic syndrome10.2
31hodgkin's lymphoma, lymphocytic-histiocytic predominance10.2CCR4, CD7, TNFRSF8
32autoimmune lymphoproliferative syndrome, type v10.2ALK, FAS, TIA1
33collagen disease10.1ALK, GZMB, NPM1, TIA1, TNFRSF8
34polycystic bone disease10.1FAS, TYK2
35chronic lymphocytic leukemia10.1
36pyoderma10.1
37alk-positive anaplastic large cell lymphoma10.0ALK, IRF4, NPM1, TRG, TYK2
38giardiasis10.0CCL17, CXCR3
39behcet's disease9.9IRF4, TIA1, TNFRSF8
40alcohol abuse9.9CD7, FAS, IRF4, TNFRSF8
41rheumatoid arthritis9.9
42multiple myeloma9.9
43arthritis9.9
44b-cell lymphomas9.9
45crohn's disease9.9
46lymphomatoid granulomatosis9.9
47peripheral t-cell lymphoma9.9
48retinal vein occlusion9.9
49skin conditions9.9
50myeloma9.9

Graphical network of the top 20 diseases related to Lymphomatoid Papulosis:



Diseases related to lymphomatoid papulosis

Symptoms for Lymphomatoid Papulosis

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UMLS symptoms related to Lymphomatoid Papulosis:


hepatomegaly

Drugs & Therapeutics for Lymphomatoid Papulosis

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Drugs for Lymphomatoid Papulosis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

idNameStatusPhaseClinical TrialsCas NumberPubChem Id
1Immunologic FactorsPhase 218483
2ImmunoglobulinsPhase 24477
3Antibodies, MonoclonalPhase 22413
4AntibodiesPhase 24477

Interventional clinical trials:

idNameStatusNCT IDPhase
1Study of SGN-30 (Anti-CD30 mAb) in Patients With Primary Cutaneous Anaplastic Large Cell LymphomaCompletedNCT00099255Phase 2
2SGN-35 in CD30-positive Lymphoproliferative Disorders (ALCL), Mycosis Fungoides (MF), and Extensive Lymphomatoid Papulosis (LyP)RecruitingNCT01352520Phase 2

Search NIH Clinical Center for Lymphomatoid Papulosis

Genetic Tests for Lymphomatoid Papulosis

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Anatomical Context for Lymphomatoid Papulosis

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MalaCards organs/tissues related to Lymphomatoid Papulosis:

33
Skin, T cells, Breast, Heart, Prostate, B cells, Liver

Animal Models for Lymphomatoid Papulosis or affiliated genes

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MGI Mouse Phenotypes related to Lymphomatoid Papulosis:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00020068.3CXCR3, FAS, FASLG, IRF4, JUNB, NPM1
2MP:00053888.2ACVRL1, CCR3, CCR4, CXCR3, FAS, FASLG
3MP:00053678.1ACVRL1, CLU, CXCR3, FAS, FASLG, JUNB
4MP:00053975.4ACVRL1, CCL17, CCR3, CCR4, CD7, CXCR3
5MP:00053875.0CCL17, CCR3, CCR4, CD7, CLU, CXCR3

Publications for Lymphomatoid Papulosis

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Articles related to Lymphomatoid Papulosis:

(show top 50)    (show all 386)
idTitleAuthorsYear
1
Type D lymphomatoid papulosis simulating aggressive epidermotropic cytotoxic lymphoma. (26960644)
2016
2
Lymphomatoid papulosis associated with follicular B-cell lymphoma in lymph nodes. (26282183)
2015
3
Lymphomatoid papulosis in a young adult of African descent. (27051732)
2015
4
Lymphomatoid papulosis as a harbinger of chronic lymphocytic leukemia. (24668491)
2014
5
Tattoos and coincidental skin conditions: the example of lymphomatoid papulosis. (24841722)
2014
6
Association of clinicopathological characteristics with secondary neoplastic lymphoproliferative disorders in patients with lymphomatoid papulosis. (25242096)
2014
7
Steroid-Resistant Localized Lymphomatoid Papulosis Treated with Local Bath-PUVA Therapy. (23716855)
2013
8
Regional lymphomatoid papulosis successfully controlled by interferon I+-2b and nitrogen mustard solution. (23981641)
2013
9
A rare case of ulcerative proctitis associated with type B lymphomatoid papulosis and superimposed human cytomegalovirus infection. (22715829)
2012
10
EORTC, ISCL, and USCLC consensus recommendations for the treatment of primary cutaneous CD30-positive lymphoproliferative disorders: lymphomatoid papulosis and primary cutaneous anaplastic large-cell lymphoma. (21841159)
2011
11
Persistent agmination of lymphomatoid papulosis: a new case with immunohistopathologically confirmed mycosis fungoides component. (21839314)
2011
12
Type B follicular lymphomatoid papulosis. (20666192)
2010
13
Solitary oral ulceration as the first appearance of lymphomatoid papulosis: a diagnostic challenge. (19486068)
2010
14
Spectrum of CD30+ lymphoid proliferations in the eyelid lymphomatoid papulosis, cutaneous anaplastic large cell lymphoma, and anaplastic large cell lymphoma. (19969358)
2010
15
Acral lymphomatoid papulosis. (20159331)
2010
16
Investigation on clinicopathologic features of lymphomatoid papulosis]. (20078970)
2009
17
Dermoscopy of lymphomatoid papulosis. (19687447)
2009
18
Clinical, histopathologic, and immunophenotypic features of lymphomatoid papulosis with CD8 predominance in 14 pediatric patients. (19577330)
2009
19
Prognosis of lymphomatoid papulosis. (16951399)
2006
20
Three cases of lymphomatoid papulosis with a CD56+ immunophenotype. (17052504)
2006
21
Two cases of lymphomatoid papulosis in children. (12657013)
2003
22
Retinal vein occlusion in lymphomatoid papulosis. (12510725)
2002
23
Localized lymphomatoid papulosis. (11834855)
2002
24
Pseudocarcinomatous change in lymphomatoid papulosis and primary cutaneous CD30+ lymphoma: a clinicopathologic and immunohistochemical study of 6 patients. (11174381)
2001
25
Lymphomatoid papulosis with a natural killer-cell phenotype. (11531801)
2001
26
Progression of lymphomatoid papulosis to systemic lymphoma is associated with escape from growth inhibition by transforming growth factor-beta and CD30 ligand. (11594583)
2001
27
Keratoacanthomas and lymphomatoid papulosis. (9700388)
1998
28
Lymphomatoid papulosis and progression to T-cell immunoblastic lymphoma. (9643341)
1998
29
Lymphomatoid papulosis and FK 506. (9159004)
1997
30
Is it lymphoma or lymphomatoid papulosis? (9146539)
1997
31
Absence of HTLV-1 proviral sequences in patients with lymphomatoid papulosis. (9406828)
1997
32
The dominant T cell clone is present in multiple regressing skin lesions and associated T cell lymphomas of patients with lymphomatoid papulosis. (8618007)
1996
33
Cytogenetic findings in regressing skin lesions of lymphomatoid papulosis. (7697626)
1995
34
Hydroa vacciniforme-like lymphomatoid papulosis in a Japanese child: a new subset. (7829745)
1995
35
Lymphomatoid papulosis. (8113450)
1994
36
Lymphomatoid papulosis followed by acute myeloblastic leukemia. (8391031)
1993
37
Lymphomatoid papulosis followed by large-cell lymphoma: immunophenotypical and genotypical analysis. (1645581)
1991
38
Lymphomatoid papulosis update. A historical perspective. (2679400)
1989
39
Lymphomatoid papulosis. A follow-up study of 30 patients. (2889756)
1987
40
Abnormal T-lymphocytes in lymphomatoid papulosis. A cytomorphological study with a reconstruction of a major part of the cell differentiation cycle. (2883764)
1987
41
Atypical cells in lymphomatoid papulosis express the Hodgkin cell-associated antigen Ki-1. (2427610)
1986
42
Hodgkin's disease complicating lymphomatoid papulosis. (3911804)
1985
43
DNA analysis by flow cytometry in lymphomatoid papulosis. (6641008)
1983
44
Lymphomatoid papulosis. Case report providing evidence for a monocyte-macrophage origin of the atypical cells. (6883285)
1983
45
Lymphomatoid papulosis. Macaulay's disease. (7270814)
1981
46
Cutaneous lesions of lymphomatoid granulomatosis. Comparison with lymphomatoid papulosis. (7270809)
1981
47
Lymphomatoid papulosis and other pseudomalignancies of the skin. (576506)
1977
48
Lymphomatoid papulosis eventuating in mycosis fungoides. (4479691)
1974
49
Lymphomatoid papulosis. An electron microscope study of the acute and healing stages with demonstration of paramyxovirus-like particles. (4117077)
1972
50

Variations for Lymphomatoid Papulosis

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Cosmic variations for Lymphomatoid Papulosis:

7
id Cosmic Mut ID Gene Symbol COSMIC Disease Classification
(Primary site, Site subtype, Primary histology, Histology subtype)
Conf
1COSM10812TP53haematopoietic and lymphoid tissue,skin,lymphoid neoplasm,lymphomatoid papulosis6

Expression for genes affiliated with Lymphomatoid Papulosis

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Search GEO for disease gene expression data for Lymphomatoid Papulosis.

Pathways for genes affiliated with Lymphomatoid Papulosis

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Pathways related to Lymphomatoid Papulosis according to GeneCards Suite gene sharing:

(show all 12)
idSuper pathwaysScoreTop Affiliating Genes
1
Show member pathways
9.5FASLG, GZMB, TYK2
29.3FAS, FASLG, HBB
3
Show member pathways
9.3FAS, FASLG, GZMB
4
Show member pathways
9.2FASLG, IRF4, JUNB
5
Show member pathways
9.2FASLG, GZMB, JUNB
6
Show member pathways
9.0CCL17, CCR3, CCR4, CXCR3
79.0CD7, CXCR3, GZMB, IRF4
88.8FAS, FASLG, JUNB, PDGFRA
9
Show member pathways
7.4ACVRL1, CCL17, CCR3, CCR4, CXCR3, FAS
10
Show member pathways
7.0ACVRL1, ALK, CCL17, CCR3, CCR4, CXCR3
11
Show member pathways
6.7ACVRL1, ALK, CCL17, CCR3, CCR4, CXCR3
12
Show member pathways
6.7ACVRL1, ALK, CCL17, CCR3, CCR4, CXCR3

GO Terms for genes affiliated with Lymphomatoid Papulosis

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Cellular components related to Lymphomatoid Papulosis according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1apical dendriteGO:00974409.7CLU, FAS

Biological processes related to Lymphomatoid Papulosis according to GeneCards Suite gene sharing:

(show all 10)
idNameGO IDScoreTop Affiliating Genes
1type I interferon signaling pathwayGO:006033710.6IRF4, TYK2
2retina vasculature development in camera-type eyeGO:006129810.4ACVRL1, PDGFRA
3platelet aggregationGO:007052710.3HBB, PDGFRA
4necroptotic signaling pathwayGO:009752710.2FAS, FASLG
5transmembrane receptor protein tyrosine kinase signaling pathwayGO:000716910.1ALK, PDGFRA, TYK2
6response to growth factorGO:007084810.1FAS, FASLG
7necroptotic processGO:00702669.7FAS, FASLG
8cell surface receptor signaling pathwayGO:00071669.4CXCR3, FAS, FASLG
9chemokine-mediated signaling pathwayGO:00700989.0CCL17, CCR3, CCR4, CXCR3
10response to lipopolysaccharideGO:00324968.9FAS, FASLG, JUNB, TNFRSF8

Sources for Lymphomatoid Papulosis

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2CDC
14ExPASy
15FDA
16FMA
24GTR
25HGMD
26HMDB
27ICD10
28ICD10 via Orphanet
29ICD9CM
30IUPHAR
31KEGG
34MedGen
36MeSH
37MESH via Orphanet
38MGI
41NCI
42NCIt
43NDF-RT
46NINDS
47Novoseek
49OMIM
50OMIM via Orphanet
54PubMed
55QIAGEN
60SNOMED-CT via Orphanet
64Tumor Gene Family of Databases
65UMLS
66UMLS via Orphanet