LYP
MCID: LYM052
MIFTS: 45

Lymphomatoid Papulosis (LYP)

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

Aliases & Classifications for Lymphomatoid Papulosis

MalaCards integrated aliases for Lymphomatoid Papulosis:

Name: Lymphomatoid Papulosis 53 59 55 73
Lyp 53 59

Characteristics:

Orphanet epidemiological data:

59
lymphomatoid papulosis
Inheritance: Not yet documented; Age of onset: All ages; Age of death: normal life expectancy;

Classifications:



External Ids:

Orphanet 59 ORPHA98842
MESH via Orphanet 45 D017731
UMLS via Orphanet 74 C0206182
ICD10 via Orphanet 34 C86.6
ICD10 33 L41.2
UMLS 73 C0206182

Summaries for Lymphomatoid Papulosis

NIH Rare Diseases : 53 Lymphomatoid papulosis (LyP) is a non-contagious, chronic skin condition characterized by the eruption of recurring, self-healing bumps (lesions) on the skin. The lesions typically begin small and then become larger, and they may bleed or ulcerate before becoming scaly and crusty. They often develop a red-brown color. Symptoms associated with lesions may include itching and/or pain, which may be debilitating. The frequency of eruptions varies over time and from person to person. Lesions may be very persistent, or they may go away for long periods of time before coming back. They may occur anywhere on the body, but they typically develop on the trunk, arms, and legs, and may develop on the hands, face, and genitalia. They generally go away on their own over a period of weeks to months (usually between 3 and 8 weeks), with or without scarring. LyP is not contagious. The cause of LyP is not known, but there is no evidence that it is hereditary. Stress is often reported to trigger eruptions of lesions. The duration of the condition varies from person to person and may last months, years or decades. A diagnosis of LyP requires evaluating the symptoms and having a skin biopsy for various types of laboratory tests. While LyP usually is not classified as a cancer (although there has been some debate), it has characteristics of lymphoma under the microscope, and people with LyP have a life-long increased risk of developing lymphoma such as mycosis fungoides, PC-ALCL, or Hodgkin lymphoma. In 5 to 20 percent of people with LyP, the condition is either preceded by lymphoma, associated with lymphoma, or followed by lymphoma. When LyP is diagnosed it is important to rule out these cancers, and for this reason, various blood tests or imaging studies may also be recommended. Treatment for LyP may speed up the healing of existing lesions or prevent new lesions from forming, but it does not change the overall course or duration of the condition. People with only a few lesions or with no major symptoms or cosmetic concerns may opt to forego treatment. If treatment is desired, topical corticosteroids are an option. Options for people with extensive skin lesions or debilitating symptoms may include topical steroids, phototherapy, oral or topical retinoids, methotrexate, or other medications (alone or in combination).Tetracycline is typically used if ulcerated lesions become infected. In most people with LyP-associated lymphomas, treatment of the lymphoma will also clear the LyP. The long-term outlook (prognosis) in more than 90% of people with LyP is positive, as it usually does not affect overall health. People with LyP who do not develop cancer have a normal life expectancy. While there is a substantial risk to develop lymphoma, the reported mortality rates from associated lymphomas are low.

MalaCards based summary : Lymphomatoid Papulosis, also known as lyp, is related to anaplastic large cell lymphoma and autoimmune lymphoproliferative syndrome, type v. An important gene associated with Lymphomatoid Papulosis is NPM1 (Nucleophosmin 1), and among its related pathways/superpathways are Apoptosis Modulation and Signaling and Influenza A. The drugs Lenalidomide and Immunologic Factors have been mentioned in the context of this disorder. Affiliated tissues include skin, t cells and testes, and related phenotypes are Synthetic lethal with MLN4924 (a NAE inhibitor) and Synthetic lethal with MLN4924 (a NAE inhibitor)

Wikipedia : 76 Lymphomatoid papulosis (LyP) is a rare skin disorder. The overall prevalence rate of lymphomatoid... more...

Related Diseases for Lymphomatoid Papulosis

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

(show top 50) (show all 111)
# Related Disease Score Top Affiliating Genes
1 anaplastic large cell lymphoma 31.1 ALK GZMB NPM1
2 autoimmune lymphoproliferative syndrome, type v 30.5 ALK FAS TIA1
3 cutaneous t cell lymphoma 30.5 GZMB TIA1
4 lymphomatoid granulomatosis 30.2 GZMB TIA1
5 primary cutaneous anaplastic large cell lymphoma 30.2 ALK GZMB NPM1 TIA1 TYK2
6 lymphopenia 30.1 FAS FASLG
7 peripheral t-cell lymphoma 30.0 ALK GZMB TIA1
8 lymphoproliferative syndrome, x-linked, 1 11.5
9 lymphoma 10.9
10 mycosis fungoides 10.7
11 pityriasis lichenoides 10.6
12 pityriasis lichenoides et varioliformis acuta 10.4
13 histiocytosis 10.4
14 hypereosinophilic syndrome 10.4
15 autoimmune disease 10.3
16 leukemia, chronic lymphocytic 2 10.3
17 leukemia, chronic lymphocytic 10.3
18 leukemia 10.3
19 lymphocytic leukemia 10.3
20 leukemia, b-cell, chronic 10.3
21 autoimmune disease 1 10.2
22 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.2
23 multiple sclerosis 10.2
24 myeloma, multiple 10.2
25 b-cell lymphomas 10.2
26 ichthyosis 10.2
27 pyoderma 10.2
28 cll/sll 10.2
29 ichthyosis, acquired 10.2
30 alk-positive anaplastic large cell lymphoma 10.1 ALK NPM1
31 lung lymphoma 10.1 ALK GZMB
32 orbit lymphoma 10.1 GZMB TIA1
33 lichen sclerosus et atrophicus 10.1 GZMB TIA1
34 viral esophagitis 10.1 GZMB TIA1
35 subcutaneous panniculitis-like t-cell lymphoma 10.1 GZMB TIA1
36 composite lymphoma 10.1 GZMB TIA1
37 hepatosplenic t-cell lymphoma 10.1 GZMB TIA1
38 natural killer cell leukemia 10.1 GZMB TIA1
39 giardiasis 10.1 GZMB TIA1
40 infiltrating lipoma 10.1 GZMB TIA1
41 necrotizing sialometaplasia 10.1 GZMB TIA1
42 ileum cancer 10.0 GZMB TIA1
43 angioimmunoblastic t-cell lymphoma 10.0 GZMB TIA1
44 inflammatory myofibroblastic tumor 10.0 ALK NPM1
45 cowpox 10.0 FAS GZMB
46 rheumatoid arthritis 10.0
47 langerhans cell histiocytosis 10.0
48 basal cell carcinoma 1 10.0
49 arthritis 10.0
50 crohn's disease 10.0

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



Diseases related to Lymphomatoid Papulosis

Symptoms & Phenotypes for Lymphomatoid Papulosis

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

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Synthetic lethal with MLN4924 (a NAE inhibitor) GR00250-A-1 9.17 FAS GZMB TIA1
2 Synthetic lethal with MLN4924 (a NAE inhibitor) GR00250-A-2 9.17 FAS FASLG GZMB TIA1

MGI Mouse Phenotypes related to Lymphomatoid Papulosis:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 neoplasm MP:0002006 9.26 ALK FAS FASLG NPM1
2 skeleton MP:0005390 9.02 ALK FAS FASLG TIA1 TYK2

Drugs & Therapeutics for Lymphomatoid Papulosis

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

(show all 10)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Lenalidomide Approved Phase 2 191732-72-6 216326
2 Immunologic Factors Phase 2
3 Immunoglobulins Phase 2
4 Antibodies, Monoclonal Phase 2
5 Antibodies Phase 2
6 Anti-Bacterial Agents Phase 2
7 Angiogenesis Modulating Agents Phase 2
8 Anti-Infective Agents Phase 2
9 Angiogenesis Inhibitors Phase 2
10 Immunosuppressive Agents Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Study of SGN-30 (Anti-CD30 mAb) in Patients With Primary Cutaneous Anaplastic Large Cell Lymphoma Completed NCT00099255 Phase 2 SGN-30
2 Dosing of Brentuximab Vedotin for Mycosis Fungoides Patients Recruiting NCT03587844 Phase 2 brentuximab vedotin;brentuximab vedotin;brentuximab vedotin
3 Brentuximab Vedotin and Lenalidomide in Treating Patients With Stage IB-IVB Relapsed or Refractory T-Cell Lymphoma Recruiting NCT03409432 Phase 2 Brentuximab Vedotin;Lenalidomide
4 SGN-35 in CD30-positive Lymphoproliferative Disorders (ALCL), Mycosis Fungoides (MF), and Extensive Lymphomatoid Papulosis (LyP) Active, not recruiting NCT01352520 Phase 2 SGN-35
5 Tissue Repository: CTCL Collection Protocol Recruiting NCT02840747

Search NIH Clinical Center for Lymphomatoid Papulosis

Genetic Tests for Lymphomatoid Papulosis

Anatomical Context for Lymphomatoid Papulosis

MalaCards organs/tissues related to Lymphomatoid Papulosis:

41
Skin, T Cells, Testes, B Cells, Breast, Lung, Thyroid

Publications for Lymphomatoid Papulosis

Articles related to Lymphomatoid Papulosis:

(show top 50) (show all 462)
# Title Authors Year
1
Lymphomatoid papulosis with folliculotropism, eccrinotropism and neurotropism. ( 29660166 )
2018
2
Lymphomatoid papulosis presenting as a rash in a patient with HIV infection. ( 29665741 )
2018
3
Type D (CD8+) lymphomatoid papulosis in a patient with classic (CD4+) mycosis fungoides. ( 29378678 )
2018
4
Lymphomatoid Papulosis (LyP) Associated with Diffuse Large B-cell Lymphoma and Cutaneous Anaplastic Large Cell Lymphoma: LyP as a Haematological Malignancy-related Dermadrome. ( 29796688 )
2018
5
Successful control of phototherapy-resistant lymphomatoid papulosis with oral bexarotene. ( 28971510 )
2018
6
First-line treatment in lymphomatoid papulosis: a retrospective multicentre study. ( 28994134 )
2018
7
Unilesional locally recurrent lymphomatoid papulosis with variable histological presentation. ( 28562138 )
2018
8
Lymphomatoid Contact Dermatitis Induced by Acrylates Mimicking Lymphomatoid Papulosis. ( 29570488 )
2018
9
Lymphomatoid papulosis in patients with chronic lymphocytic leukemia/small lymphocytic lymphoma: case report and literature review. ( 30142733 )
2018
10
Lymphomatoid papulosis associated with chronic lymphocytic leukaemia/small lymphocytic lymphoma: three cases. ( 28569995 )
2018
11
Clinical evolution of lymphomatoid papulosis. ( 29808923 )
2018
12
A case of lymphomatoid papulosis, pityriasis lichenoides acuta, and mycosis fungoides coexistence. ( 30198062 )
2018
13
The Alphabetization of Lymphomatoid Papulosis: Focus on "F". ( 30207529 )
2018
14
Type B lymphomatoid papulosis. ( 30374384 )
2018
15
Long-term Follow-up of a Case of Lymphomatoid Papulosis with a Benign Course. ( 30390731 )
2018
16
CD8-positive lymphomatoid papulosis (type D): Some lesions may lack CD30 expression and overlap histologically with mycosis fungoides. ( 30520526 )
2018
17
Molecular dynamics, thermodynamic, and mutational binding studies for tumor-specific LyP-1 in complex with p32. ( 28427307 )
2018
18
LyP-1 Conjugated Nanoparticles for Magnetic Resonance Imaging of Triple Negative Breast Cancer. ( 29101636 )
2018
19
LyP-1-conjugated Fe3O4 nanoparticles suppress tumor growth by magnetic induction hyperthermia. ( 29165044 )
2018
20
Structure Reconstruction of LyP-1: Lc(LyP-1) Coupling by Amide Bond Inspires the Brain Metastatic Tumor Targeted Drug Delivery. ( 29215294 )
2018
21
Correction to: LyP-1 Conjugated Nanoparticles for Magnetic Resonance Imaging of Triple Negative Breast Cancer. ( 29556946 )
2018
22
Oral Lymphomatoid papulosis type C: A diagnostic pitfall, often confused with T-cell lymphoma. ( 29146059 )
2017
23
Dermoscopy of different stages of lymphomatoid papulosis. ( 29171911 )
2017
24
A Case of Lymphomatoid Papulosis Type E With an Unusual Exacerbated Clinical Course. ( 28937425 )
2017
25
Imatinib Treatment of Lymphomatoid Papulosis Associated with Myeloproliferative Hypereosinophilic Syndrome Presenting the FIP1L1-PDGFRA Fusion Gene. ( 28374041 )
2017
26
A Severe Case of Lymphomatoid Papulosis Type E Successfully Treated with Interferon-Alfa 2a. ( 28540091 )
2017
27
An unusual case of lymphomatoid papulosis type E with extensive necrosis. ( 29067932 )
2017
28
Lymphomatoid papulosis - making sense of the alphabet soup: a proposal to simplify terminology. ( 28252259 )
2017
29
Associated Hematolymphoid Malignancies in Patients With Lymphomatoid Papulosis: A Canadian Retrospective Study. ( 28614957 )
2017
30
Brentuximab Vedotin for Patients With Refractory Lymphomatoid Papulosis: An Analysis of Phase 2 Results. ( 28980004 )
2017
31
Magnetic mesoporous nanospheres anchored with LyP-1 as an efficient pancreatic cancer probe. ( 27871003 )
2017
32
Design and In Vitro Evaluation of Bispecific Complexes and Drug Conjugates of Anticancer Peptide, LyP-1 in Human Breast Cancer. ( 27896591 )
2017
33
Histopathological aspects and differential diagnosis of CD8 positive lymphomatoid papulosis. ( 27461449 )
2016
34
Lymphomatoid Papulosis in a 2-Year-Old Girl With Markedly Elevated Antinuclear Antibodies. ( 28006986 )
2016
35
Human T-cell leukemia virus type 1-positive lymphomatoid papulosis. ( 26771231 )
2016
36
Lymphomatoid papulosis associated with multiple myeloma. ( 27230953 )
2016
37
Type D lymphomatoid papulosis simulating aggressive epidermotropic cytotoxic lymphoma. ( 26960644 )
2016
38
Lymphomatoid papulosis and fingolimod-A new connection? ( 26993118 )
2016
39
Regional lymphomatoid papulosis of the breast restricted to an area of prior radiotherapy. ( 27274553 )
2016
40
Lymphomatoid Papulosis Type D: Report of a Case in a Child and Review of the Literature. ( 26763888 )
2016
41
Pseudocarcinomatous Hyperplasia Hiding Lymphomatoid Papulosis: A "Low-Power View" Pitfall. ( 26711583 )
2016
42
Follicular lymphomatoid papulosis with follicular mucinosis: a clinicopathologic study of 3 cases with literature review and conceptual reappraisal. ( 28009441 )
2016
43
Follicular Lymphomatoid Papulosis: An Eosinophilic-Rich Follicular Subtype Masquerading as Folliculitis Clinically and Histologically. ( 26730698 )
2016
44
Lymphomatoid Papulosis in Children and Adolescents: A Systematic Review. ( 27138554 )
2016
45
Angioinvasive lymphomatoid papulosis (typeA E): a first Japanese case. ( 27267092 )
2016
46
A child with mastocytosis and lymphomatoid papulosis. ( 27190619 )
2016
47
Frequency and Risk Factors for Associated Lymphomas in Patients With Lymphomatoid Papulosis. ( 26668250 )
2016
48
Lymphomatoid papulosis type D in a fingolimod-treated multiple sclerosis patient. ( 27053634 )
2016
49
Shared clonality in distinctive lesions of lymphomatoid papulosis and mycosis fungoides occurring in the same patients suggests a common origin. ( 25666664 )
2015
50
Results of a Phase II Trial of Brentuximab Vedotin for CD30+ Cutaneous T-Cell Lymphoma and Lymphomatoid Papulosis. ( 26261247 )
2015

Variations for Lymphomatoid Papulosis

Expression for Lymphomatoid Papulosis

Search GEO for disease gene expression data for Lymphomatoid Papulosis.

Pathways for Lymphomatoid Papulosis

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

(show all 18)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.45 FAS FASLG GZMB
2
Show member pathways
12.43 FAS FASLG TYK2
3
Show member pathways
12.34 FAS FASLG NPM1
4
Show member pathways
12.13 FAS FASLG GZMB
5
Show member pathways
12.09 FAS FASLG GZMB
6
Show member pathways
11.8 FAS FASLG GZMB
7 11.77 FAS FASLG GZMB TYK2
8
Show member pathways
11.74 FAS FASLG TYK2
9 11.6 ALK NPM1 TYK2
10
Show member pathways
11.48 FAS FASLG
11 11.41 FAS FASLG
12
Show member pathways
11.38 FASLG GZMB TYK2
13
Show member pathways
11.31 FASLG GZMB
14 11.22 FAS FASLG
15 11.19 FAS FASLG GZMB TIA1
16 11.14 FAS FASLG
17 10.98 FAS FASLG
18 10.95 TIA1 TYK2

GO Terms for Lymphomatoid Papulosis

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

# Name GO ID Score Top Affiliating Genes
1 apoptotic process GO:0006915 9.46 FAS FASLG GZMB TIA1
2 activation of cysteine-type endopeptidase activity involved in apoptotic process GO:0006919 9.4 FAS FASLG
3 apoptotic signaling pathway GO:0097190 9.37 FAS FASLG
4 extrinsic apoptotic signaling pathway GO:0097191 9.32 FAS FASLG
5 negative regulation of extrinsic apoptotic signaling pathway via death domain receptors GO:1902042 9.26 FAS FASLG
6 regulation of extrinsic apoptotic signaling pathway via death domain receptors GO:1902041 8.96 FAS FASLG
7 necroptotic signaling pathway GO:0097527 8.62 FAS FASLG

Sources for Lymphomatoid Papulosis

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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