MCID: LYM052
MIFTS: 41

Lymphomatoid Papulosis malady

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

Aliases & Classifications for Lymphomatoid Papulosis

About this section
Sources:
30ICD10, 31ICD10 via Orphanet, 40MESH via Orphanet, 48NIH Rare Diseases, 50Novoseek, 54Orphanet, 68UMLS, 69UMLS via Orphanet
See all MalaCards sources

Aliases & Descriptions for Lymphomatoid Papulosis:

Name: Lymphomatoid Papulosis 48 54 50 68
Lyp 48 54
 
X-Linked Lymphoproliferative Disorder 68

Characteristics:

Orphanet epidemiological data:

54
lymphomatoid papulosis:
Inheritance: Not yet documented; Age of onset: All ages; Age of death: normal life expectancy

Classifications:



External Ids:

Orphanet54 ORPHA98842
MESH via Orphanet40 D017731
UMLS via Orphanet69 C0206182
ICD10 via Orphanet31 C86.6
ICD1030 L41.2

Summaries for Lymphomatoid Papulosis

About this section
NIH Rare Diseases:48 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 eosinophilia and lymphoma, and has symptoms including hepatomegaly An important gene associated with Lymphomatoid Papulosis is NPM1 (Nucleophosmin), and among its related pathways are IL-10 Pathway and Downstream signaling in naive CD8+ T cells. Affiliated tissues include t cells, skin and bone marrow, and related mouse phenotypes are Synthetic lethal with MLN4924 (a NAE inhibitor) and neoplasm.

Wikipedia:71 Lymphomatoid papulosis (LyP) is a rare skin disorder. The overall prevalence rate of lymphomatoid... more...

Related Diseases for Lymphomatoid Papulosis

About this section

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

(show top 50)    (show all 89)
idRelated DiseaseScoreTop Affiliating Genes
1eosinophilia30.0GZMB, TIA1
2lymphoma10.8
3anaplastic large cell lymphoma10.5
4mycosis fungoides10.5
5pityriasis lichenoides10.4
6congenital sucrase-isomaltase deficiency with starch intolerance10.3ALK, NPM1
7cutaneous t cell lymphoma10.3
8male infertility10.2GZMB, TIA1
9subependymal nodular heterotopia10.2GZMB, TIA1
10primary cutaneous anaplastic large cell lymphoma10.2
11pseudotyphus of california10.2GZMB, TIA1
12clivus chordoma10.2GZMB, TIA1
13gray zone lymphoma10.2GZMB, TIA1
14pityriasis lichenoides et varioliformis acuta10.2
15breast fibroadenosis10.2GZMB, TIA1
16renal hypertension10.2GZMB, TIA1
17lumbosacral lipoma10.2GZMB, TIA1
18mixed cell type cancer10.2GZMB, TIA1
19lacrimal passage granuloma10.2GZMB, TIA1
20sialolithiasis10.1GZMB, TIA1
21histiocytosis10.1
22lymphopenia10.1
23leukemia10.1
24hypereosinophilic syndrome10.1
25tonsillar fossa cancer10.1FAS, GZMB
26benign adult familial myoclonic epilepsy10.0FAS, TYK2
27mixed extragonadal germ cell cancer10.0ALK, NPM1
28trigeminal nerve disease10.0FASLG, GZMB
29polycystic ovary syndrome9.9ALK, GZMB, TIA1
30multiple myeloma9.9
31chronic lymphocytic leukemia9.9
32pyoderma9.9
33hemolytic anemia9.9ALK, NPM1
34bolivian hemorrhagic fever9.9FASLG, GZMB
3512p12.1 microdeletion syndrome9.8FAS, FASLG
36hypodermyiasis9.8FASLG, GZMB
37pediatric systemic lupus erythematosus9.8FAS, FASLG
38rheumatoid arthritis9.8
39arthritis9.8
40b-cell lymphomas9.8
41crohn's disease9.8
42lymphomatoid granulomatosis9.8
43peripheral t-cell lymphoma9.8
44retinal vein occlusion9.8
45skin conditions9.8
46basal cell carcinoma9.8
47transitional cell carcinoma9.8
48proctitis9.8
49keratoacanthoma9.8
50thymoma9.8

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



Diseases related to lymphomatoid papulosis

Symptoms & Phenotypes for Lymphomatoid Papulosis

About this section

UMLS symptoms related to Lymphomatoid Papulosis:


hepatomegaly

GenomeRNAi Phenotypes related to Lymphomatoid Papulosis according to GeneCards Suite gene sharing:

26
idDescriptionGenomeRNAi Source AccessionScoreTop Affiliating Genes
1GR00250-A-18.0FAS, FASLG, GZMB, TIA1

MGI Mouse Phenotypes related to Lymphomatoid Papulosis according to GeneCards Suite gene sharing:

41
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00020069.1ALK, FAS, FASLG, NPM1
2MP:00053908.5ALK, FAS, FASLG, TIA1, TYK2

Drugs & Therapeutics for Lymphomatoid Papulosis

About this section

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

idNameStatusPhaseClinical TrialsCas NumberPubChem Id
1ImmunoglobulinsPhase 26045
2Antibodies, MonoclonalPhase 23795
3AntibodiesPhase 26045

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)Active, not recruitingNCT01352520Phase 2
3Tissue Repository: CTCL Collection ProtocolNot yet recruitingNCT02840747

Search NIH Clinical Center for Lymphomatoid Papulosis

Genetic Tests for Lymphomatoid Papulosis

About this section

Anatomical Context for Lymphomatoid Papulosis

About this section

MalaCards organs/tissues related to Lymphomatoid Papulosis:

36
T cells, Skin, Bone marrow, Breast, Bone, B cells, Tongue

Publications for Lymphomatoid Papulosis

About this section

Articles related to Lymphomatoid Papulosis:

(show top 50)    (show all 391)
idTitleAuthorsYear
1
Type D lymphomatoid papulosis simulating aggressive epidermotropic cytotoxic lymphoma. (26960644)
2016
2
Histopathological aspects and differential diagnosis of CD8 positive lymphomatoid papulosis. (27461449)
2016
3
Lymphomatoid Papulosis in Children and Adolescents: A Systematic Review. (27138554)
2016
4
Lymphomatoid Papulosis Type D: Report of a Case in a Child and Review of the Literature. (26763888)
2016
5
Lymphomatoid papulosis and fingolimod-A new connection? (26993118)
2016
6
Angioinvasive lymphomatoid papulosis (typeA E): a first Japanese case. (27267092)
2016
7
Lymphomatoid papulosis associated with multiple myeloma. (27230953)
2016
8
Lymphomatoid Papulosis in a 2-Year-Old Girl With Markedly Elevated Antinuclear Antibodies. (28006986)
2016
9
Pseudocarcinomatous Hyperplasia Hiding Lymphomatoid Papulosis: A "Low-Power View" Pitfall. (26711583)
2016
10
Regional lymphomatoid papulosis of the breast restricted to an area of prior radiotherapy. (27274553)
2016
11
Follicular Lymphomatoid Papulosis: An Eosinophilic-Rich Follicular Subtype Masquerading as Folliculitis Clinically and Histologically. (26730698)
2016
12
Human T-cell leukemia virus type 1-positive lymphomatoid papulosis. (26771231)
2016
13
Follicular lymphomatoid papulosis with follicular mucinosis: a clinicopathologic study of 3 cases with literature review and conceptual reappraisal. (28009441)
2016
14
Frequency and Risk Factors for Associated Lymphomas in Patients With Lymphomatoid Papulosis. (26668250)
2016
15
Lymphomatoid papulosis type D in a fingolimod-treated multiple sclerosis patient. (27053634)
2016
16
A child with mastocytosis and lymphomatoid papulosis. (27190619)
2016
17
Lymphomatoid papulosis in a young adult of African descent. (27051732)
2015
18
Recommendations for treatment of lymphomatoid papulosis with methotrexate: a report from the Dutch Cutaneous Lymphoma Group. (25998985)
2015
19
New variant lymphomatoid papulosis type E preceding and coexisting with mycosis fungoides - a case report and review of the literature. (26272365)
2015
20
Localized lymphomatoid papulosis. (25556677)
2015
21
Lymphomatoid papulosis: Treatment response and associated lymphomas in a study of 180 patients. (26518172)
2015
22
Self-healing Eyelid Nodule: A Quiz. Diagnosis: Lymphomatoid papulosis (LyP), type A. (25366062)
2015
23
Lymphomatoid papulosis with pseudocarcinomatous hyperplasia in a seven-year-old girl: a case report. (26531106)
2015
24
Results of a Phase II Trial of Brentuximab Vedotin for CD30+ Cutaneous T-Cell Lymphoma and Lymphomatoid Papulosis. (26261247)
2015
25
Lymphomatoid papulosis associated with follicular B-cell lymphoma in lymph nodes. (26282183)
2015
26
Commentary about papular mycosis fungoides, lymphomatoid papulosis, and lymphomatoid pityriasis lichenoides: More similarities than differences. (26566599)
2015
27
Atypical cutaneous I^I' T cell proliferation with morphologic features of lymphoma but with clinical features and course of PLEVA or lymphomatoid papulosis. (26263922)
2015
28
Lymphomatoid papulosis: A cutaneous lymphoproliferative disorder in a patient on fingolimod for multiple sclerosis. (26219664)
2015
29
Shared clonality in distinctive lesions of lymphomatoid papulosis and mycosis fungoides occurring in the same patients suggests a common origin. (25666664)
2015
30
The efficacy of methotrexate for lymphomatoid papulosis. (25981010)
2015
31
Lymphomatoid papulosis as a harbinger of chronic lymphocytic leukemia. (24668491)
2014
32
Tattoos and coincidental skin conditions: the example of lymphomatoid papulosis. (24841722)
2014
33
Association of clinicopathological characteristics with secondary neoplastic lymphoproliferative disorders in patients with lymphomatoid papulosis. (25242096)
2014
34
A Unique Case of Concurrent Chronic Lymphocytic Leukemia / Small Lymphocytic Lymphoma and Lymphomatoid Papulosis in the Same Biopsy. (25382820)
2014
35
Intralymphatic cutaneous anaplastic large cell lymphoma/lymphomatoid papulosis: expanding the spectrum of CD30-positive lymphoproliferative disorders. (24805854)
2014
36
Lymphomatoid papulosis with 6p25.3 rearrangement: a further case of the newly described variant. (24804939)
2014
37
Lymphomatoid papulosis misdiagnosed as pityriasis lichenoides et varioliformis acuta: Two case reports and a literature review. (25371758)
2014
38
CD30-Negative Lymphomatoid Papulosis Type D in an Elderly Man. (24394303)
2014
39
Lymphomatoid papulosis type C of the eyelid in a young girl: a case report and review of literature. (24911471)
2014
40
Lymphomatoid papulosis in children: a series of 25 cases. (24749749)
2014
41
Recurrent course and CD30 expression of atypical T lymphocytes distinguish lymphomatoid papulosis from primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma. (24549273)
2014
42
Unusual CD8 positive lymphomatoid papulosis in childhood. (25419749)
2014
43
Lymphomatoid papulosis and recurrent transitional cell carcinoma of the bladder: a paraneoplastic association. (24746306)
2014
44
The importance of histopathology findings in lymphomatoid papulosis. (25611293)
2014
45
Exacerbation of lymphomatoid papulosis during rituximab therapy. (23190403)
2014
46
CD30-positive lymphoproliferative disorder in a red tattoo: regional lymphomatoid papulosis type C or pseudolymphoma? (24689991)
2014
47
Regional lymphomatoid papulosis in association with pseudoepitheliomatous hyperplasia: 13A years follow-up. (24841636)
2014
48
Comparison of CD163+ Macrophages and CD206+ Cells in Lesional Skin of CD30+ Lymphoproliferative Disorders of Lymphomatoid Papulosis and Primary Cutaneous Anaplastic Large-cell Lymphoma. (25403560)
2014
49
I^I' T cell-rich variants of pityriasis lichenoides and lymphomatoid papulosis: benign cutaneous disorders to be distinguished from aggressive cutaneous I^I' T cell lymphomas. (25143223)
2014
50
Regional lymphomatoid papulosis successfully controlled by interferon I+-2b and nitrogen mustard solution. (23981641)
2013

Variations for Lymphomatoid Papulosis

About this section

Expression for genes affiliated with Lymphomatoid Papulosis

About this section
Search GEO for disease gene expression data for Lymphomatoid Papulosis.

Pathways for genes affiliated with Lymphomatoid Papulosis

About this section

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

(show all 15)
idSuper pathwaysScoreTop Affiliating Genes
19.5TIA1, TYK2
2
Show member pathways
9.3FASLG, GZMB
39.2ALK, NPM1, TYK2
49.1FAS, FASLG
59.1FAS, FASLG
6
Show member pathways
8.9FASLG, GZMB, TYK2
7
Show member pathways
8.8FAS, FASLG, NPM1
8
Show member pathways
8.5FAS, FASLG, GZMB
9
Show member pathways
8.5FAS, FASLG, GZMB
10
Show member pathways
8.5FAS, FASLG, GZMB
11
Show member pathways
8.5FAS, FASLG, GZMB
12
Show member pathways
8.5FAS, FASLG, GZMB
13
Show member pathways
8.5FAS, FASLG, GZMB
148.1FAS, FASLG, GZMB, TYK2
158.0FAS, FASLG, GZMB, TIA1

GO Terms for genes affiliated with Lymphomatoid Papulosis

About this section

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

(show all 12)
idNameGO IDScoreTop Affiliating Genes
1activation of cysteine-type endopeptidase activity involved in apoptotic processGO:00069199.9FAS, FASLG
2activation of cysteine-type endopeptidase activity involved in apoptotic signaling pathwayGO:00972969.9FAS, FASLG
3apoptotic signaling pathwayGO:00971909.9FAS, FASLG
4extrinsic apoptotic signaling pathwayGO:00971919.9FAS, FASLG
5extrinsic apoptotic signaling pathway via death domain receptorsGO:00086259.9FAS, FASLG
6inflammatory cell apoptotic processGO:00069259.9FAS, FASLG
7necroptotic signaling pathwayGO:00975279.9FAS, FASLG
8negative regulation of extrinsic apoptotic signaling pathway via death domain receptorsGO:19020429.9FAS, FASLG
9regulation of extrinsic apoptotic signaling pathway via death domain receptorsGO:19020419.9FAS, FASLG
10response to growth factorGO:00708489.9FAS, FASLG
11apoptotic processGO:00069158.7FAS, FASLG, GZMB, TIA1
12signal transductionGO:00071658.3ALK, FAS, FASLG, NPM1

Sources for Lymphomatoid Papulosis

About this section
2CDC
6CNVD
10DGIdb
15ExPASy
16FDA
17FMA
27GTR
28HGMD
29HMDB
30ICD10
31ICD10 via Orphanet
32ICD9CM
33IUPHAR
34KEGG
37MedGen
39MeSH
40MESH via Orphanet
41MGI
44NCI
45NCIt
46NDF-RT
49NINDS
50Novoseek
52OMIM
53OMIM via Orphanet
57PubMed
58QIAGEN
63SNOMED-CT via Orphanet
67Tumor Gene Family of Databases
68UMLS
69UMLS via Orphanet