LYP
MCID: LYM052
MIFTS: 36

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 52 58 54 17 71 32
Lyp 52 58

Characteristics:

Orphanet epidemiological data:

58
lymphomatoid papulosis
Inheritance: Not yet documented; Age of onset: All ages; Age of death: normal life expectancy;

Classifications:

Orphanet: 58  
Rare skin diseases
Rare haematological diseases


External Ids:

ICD10 32 L41.2
MESH via Orphanet 44 D017731
ICD10 via Orphanet 33 C86.6
UMLS via Orphanet 72 C0206182
Orphanet 58 ORPHA98842
UMLS 71 C0206182

Summaries for Lymphomatoid Papulosis

NIH Rare Diseases : 52 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 histiocytosis and mycosis fungoides. An important gene associated with Lymphomatoid Papulosis is NPM1 (Nucleophosmin 1), and among its related pathways/superpathways are Apoptosis and Autophagy and NF-kappaB Signaling. The drugs Lenalidomide and Antineoplastic Agents, Immunological have been mentioned in the context of this disorder. Affiliated tissues include t cells, skin and testes.

Wikipedia : 74 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 186)
# Related Disease Score Top Affiliating Genes
1 histiocytosis 30.8 TNFRSF8 ALK
2 mycosis fungoides 30.6 TNFRSF8 TIA1 GZMB ALK
3 lethal midline granuloma 30.1 TNFRSF8 TIA1
4 lymphomatoid granulomatosis 30.1 TIA1 GZMB
5 anaplastic large cell lymphoma 30.0 TNFRSF8 TIA1 NPM1 GZMB ALK
6 lymphoma, hodgkin, classic 29.9 TNFRSF8 TIA1 GZMB ALK
7 cutaneous t cell lymphoma 29.7 TNFRSF8 TIA1 GZMB FAS
8 autoimmune lymphoproliferative syndrome, type v 29.7 TNFRSF8 TIA1 ALK
9 peripheral t-cell lymphoma 29.7 TNFRSF8 TIA1 GZMB ALK
10 composite lymphoma 29.7 TNFRSF8 TIA1 GZMB
11 myeloma, multiple 29.5 TNFRSF8 FAS ALK
12 t-cell lymphoma, subcutaneous panniculitis-like 29.4 TNFRSF8 TIA1 GZMB ALK
13 primary cutaneous anaplastic large cell lymphoma 29.4 TYK2 TIA1 NPM1 GZMB ALK
14 leukemia, chronic myeloid 29.4 NPM1 GZMB ALK
15 adult t-cell leukemia 29.1 TNFRSF8 FAS
16 lymphoma 29.1 TNFRSF8 TIA1 NPM1 GZMB FAS ALK
17 panniculitis 29.0 TNFRSF8 TIA1 GZMB FAS
18 b-cell lymphoma 28.7 TNFRSF8 TIA1 NPM1 FAS ALK
19 lymphoma, non-hodgkin, familial 28.2 TNFRSF8 TIA1 NPM1 GZMB FAS ALK
20 lymphoproliferative syndrome, x-linked, 1 11.7
21 lymphoproliferative syndrome 10.9
22 pityriasis lichenoides 10.8
23 skin disease 10.7
24 pityriasis lichenoides et varioliformis acuta 10.6
25 autoimmune disease 10.6
26 hypereosinophilic syndrome 10.5
27 parapsoriasis 10.4
28 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.4
29 dowling-degos disease 1 10.4
30 leukemia, chronic lymphocytic 10.3
31 exanthem 10.3
32 lymphocytic leukemia 10.3
33 leukemia 10.3
34 ichthyosis 10.3
35 pustulosis of palm and sole 10.3
36 hodgkin's lymphoma, lymphocytic-histiocytic predominance 10.3
37 crohn's disease 10.3
38 psoriasis 10.3
39 follicular mucinosis 10.3
40 ichthyosis, acquired 10.3
41 pityriasis lichenoides chronica 10.3
42 rapidly involuting congenital hemangioma 10.3
43 alk-negative anaplastic large cell lymphoma 10.3
44 multiple sclerosis 10.2
45 dermatitis, atopic 10.2
46 langerhans cell histiocytosis 10.2
47 diffuse large b-cell lymphoma 10.2
48 nephrotic syndrome 10.2
49 dermatitis 10.2
50 keratoacanthoma 10.2

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



Diseases related to Lymphomatoid Papulosis

Symptoms & Phenotypes for Lymphomatoid Papulosis

Drugs & Therapeutics for Lymphomatoid Papulosis

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

(show all 13)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Lenalidomide Approved Phase 2 191732-72-6 216326
2 Antineoplastic Agents, Immunological Phase 2
3 Angiogenesis Inhibitors Phase 2
4 Antibodies, Bispecific Phase 1, Phase 2
5 Antibodies, Monoclonal Phase 2
6 Immunologic Factors Phase 2
7 Antibodies Phase 2
8 Immunoglobulins Phase 2
9
Fludarabine Approved Phase 1 21679-14-1, 75607-67-9 30751
10 Alkylating Agents Phase 1
11 Bendamustine Hydrochloride Phase 1
12 Immunosuppressive Agents Phase 1
13 Antimetabolites Phase 1

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 A Phase II Multi-Dose Study of SGN-30 (Anti-CD30 mAb) in Patients With Primary Cutaneous Anaplastic Large Cell Lymphoma, Large Cell Transformation of Mycosis Fungoides, and Lymphomatoid Papulosis Completed NCT00099255 Phase 2 SGN-30
2 Optimizing Dosing of Brentuximab Vedotin for Mycosis Fungoides, Sezary Syndrome, and Lymphomatoid Papulosis Recruiting NCT03587844 Phase 2 brentuximab vedotin;brentuximab vedotin;brentuximab vedotin
3 A Phase II Study of Brentuximab Vedotin and Lenalidomide in Relapsed and Refractory T-Cell Lymphomas Recruiting NCT03409432 Phase 2 Brentuximab Vedotin;Lenalidomide
4 Clinical and Biological Evaluation of the Novel CD30/CD16A Tetravalent Bispecific Antibody (AFM13) in Relapsed or Refractory CD30-Positive Lymphoma With Cutaneous Presentation: A Biomarker Phase Ib/IIa Study Recruiting NCT03192202 Phase 1, Phase 2 AFM13
5 Phase II Trial of Brentuximab Vedotin (SGN-35) at Dose of 1.8 mg/kg IV Every 3 Weeks in Patients With CD30-positive Lymphoproliferative Disorders (Cutaneous Anaplastic Large T-cell Lymphoma (ALCL), Mycosis Fungoides, and Extensive Lymphomatoid Papulosis (LyP) Active, not recruiting NCT01352520 Phase 2 SGN-35
6 Phase I Study of the Administration of T Lymphocytes Co-Expressing the CD30 Chimeric Antigen Receptor (CAR) and CCR4 for Relapsed/Refractory CD30+ Hodgkin Lymphoma and CD30+ Non-Hodgkin Lymphoma Recruiting NCT03602157 Phase 1 Bendamustine;Fludarabine
7 A Tissue Repository for the Collection of Samples From Patients With Cutaneous T Cell Lymphoma and Healthy Volunteers 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:

40
T Cells, Skin, Testes, B Cells, Breast, Nk Cells, Myeloid

Publications for Lymphomatoid Papulosis

Articles related to Lymphomatoid Papulosis:

(show top 50) (show all 857)
# Title Authors PMID Year
1
Clusterin expression correlates with stage and presence of large cells in mycosis fungoides. 54 61
19289586 2009
2
Clusterin expression in cutaneous CD30-positive lymphoproliferative disorders and their histologic simulants. 54 61
19220628 2009
3
Differences in survivin location and Bcl-2 expression in CD30+ lymphoproliferative disorders of the skin compared with systemic anaplastic large cell lymphomas: an immunohistochemical study. 54 61
17484779 2007
4
c-Jun expression and activation are restricted to CD30+ lymphoproliferative disorders. 54 61
17325487 2007
5
Chemokine receptor expression in cutaneous T cell and NK/T-cell lymphomas: immunohistochemical staining and in vitro chemotactic assay. 54 61
16931956 2006
6
CD30+ lymphoproliferative disorders: histopathology, differential diagnosis, new variants, and simulators. 54 61
16412214 2006
7
Polymorphism of the CD30 promoter microsatellite repressive element is associated with development of primary cutaneous lymphoproliferative disorders. 54 61
15894695 2005
8
Differential expression of thymus and activation regulated chemokine and its receptor CCR4 in nodal and cutaneous anaplastic large-cell lymphomas and Hodgkin's disease. 54 61
12181269 2002
9
Progression of lymphomatoid papulosis to systemic lymphoma is associated with escape from growth inhibition by transforming growth factor-beta and CD30 ligand. 54 61
11594583 2001
10
The T-cell chemokine receptor CXCR3 is expressed highly in low-grade mycosis fungoides. 54 61
11242798 2001
11
Distinct effects of CD30 and Fas signaling in cutaneous anaplastic lymphomas: a possible mechanism for disease progression. 54 61
11121138 2000
12
Expression of Fas and Fas-ligand in primary cutaneous T-cell lymphoma (CTCL): association between lack of Fas expression and aggressive types of CTCL. 54 61
10951138 2000
13
Primary CD30-positive cutaneous T-cell lymphomas and lymphomatoid papulosis frequently express cytotoxic proteins. 54 61
10672058 2000
14
CD30-CD30 ligand interaction in primary cutaneous CD30(+) T-cell lymphomas: A clue to the pathophysiology of clinical regression. 54 61
10556192 1999
15
Expression of cytotoxic proteins by neoplastic T cells in mycosis fungoides increases with progression from plaque stage to tumor stage disease. 54 61
10233858 1999
16
Analysis of the t(2;5) (p23;q35) translocation in CD30+ primary cutaneous lymphoproliferative disorders and Hodgkin's disease. 54 61
9638979 1998
17
Most primary cutaneous CD30-positive lymphoproliferative disorders have a CD4-positive cytotoxic T-cell phenotype. 54 61
9347791 1997
18
Absence of anaplastic lymphoma kinase (ALK) and Epstein-Barr virus gene products in primary cutaneous anaplastic large cell lymphoma and lymphomatoid papulosis. 54 61
9415224 1997
19
The monoclonal antibody ALK1 identifies a distinct morphological subtype of anaplastic large cell lymphoma associated with 2p23/ALK rearrangements. 54 61
9250148 1997
20
Amplification of genomic DNA demonstrates the presence of the t(2;5) (p23;q35) in anaplastic large cell lymphoma, but not in other non-Hodgkin's lymphomas, Hodgkin's disease, or lymphomatoid papulosis. 54 61
8781434 1996
21
Ki-1 (CD30) expression in differentiation of lymphomatoid papulosis from arthropod bite reactions. 54 61
1344811 1992
22
Frequency and prognosis of associated malignancies in 504 patients with lymphomatoid papulosis. 61
31715046 2020
23
Frequency, risk factors and prognosis of systemic haematologic malignancies, cutaneous and other neoplasms in lymphomatoid papulosis: where are we now? 61
31997496 2020
24
Lymphomatoid Papulosis. 61
31995140 2020
25
Trichoscopic features of lymphomatoid papulosis. 61
31429495 2020
26
Clinical Characteristics and Disease Course in Black Patients With Lymphomatoid Papulosis: A Case Series 61
32023015 2020
27
Lymphomatoid papulosis: an update and review. 61
31494989 2020
28
Mycosis Fungoides, Lymphomatoid Papulosis and Hodgkin's Lymphoma in the Same Patient: Apropos of a Possible Monoclonal Origin. 61
32029943 2020
29
Diagnosis of T-cell lymphoid proliferations of the skin: putting all the pieces together. 61
31664155 2020
30
Pretibial Pruritic Papular Dermatitis: A Comprehensive Clinical and Pathologic Review of Cases at a Single Institution. 61
31188140 2020
31
Pediatric cellular neurothekeoma: Seven cases and systematic review of the literature. 61
31930561 2020
32
Lymphomatoid papulosis: overview. 61
31958921 2020
33
Does Breast Implant-Associated ALCL Begin as a Lymphoproliferative Disorder? 61
31577659 2020
34
Primary Cutaneous Anaplastic Large Cell Lymphoma (pcALCL) in the Elderly and the Importance of Sport Activity Training. 61
32013101 2020
35
Type A lymphomatoid papulosis presenting as an eyelid ulcer in a young man. 61
30644803 2019
36
Comorbidities in Mycosis Fungoides and Racial Differences in Co-Existent Lymphomatoid Papulosis: A Cross-Sectional Study of 580 Patients in an Urban Tertiary Care Center. 61
31888015 2019
37
Mycosis Fungoides Associated With Lesions in the Spectrum of Primary Cutaneous CD30+ Lymphoproliferative Disorders: The Same Process or 3 Coexisting Lymphomas? 61
30946099 2019
38
A case of febrile ulceronecrotic Mucha-Habermann disease with comorbidities. 61
30860167 2019
39
Endobronchial Pseudocarcinomatous Hyperplasia Mimicking Squamous Cell Carcinoma Associated with Primary Pulmonary ALK-negative Anaplastic Large Cell Lymphoma. 61
31705818 2019
40
18F-FDG PET/CT in Lymphomatoid Papulosis Mimicking Primary Cutaneous Anaplastic Large Cell Lymphoma. 61
31246687 2019
41
Type D lymphomatoid papulosis associated with chemotherapy/chemoradiotherapy-induced severe chronic lymphocytopenia. 61
31342531 2019
42
Traumatic Ulcerative Granuloma with Stromal Eosinophilia: CD30 analysis and clonality for T cell receptor gene re-arrangement. 61
31672390 2019
43
Lymphomatoid Papulosis and Other Lymphoma-Like Diseases. 61
31466587 2019
44
Lymphomatoid Papulosis Type B in a Patient with Crohn's Disease Treated with TNF-Alpha Inhibitors Infliximab and Adalimumab. 61
31542070 2019
45
Recent Advances in Cutaneous T-cell Lymphoma: Diagnostic and Prognostic Considerations. 61
31352988 2019
46
Angiodestructive lymphomatoid papulosis lasting more than 45 years. 61
31516992 2019
47
How I treat primary cutaneous CD30+ lymphoproliferative disorders. 61
31164331 2019
48
Lymphomatoid papulosis type E with a CD56+ immunophenotype presenting with purpura-like lesions. 61
30957244 2019
49
Expression of CCR3 and CCR4 Suggests a Poor Prognosis in Mycosis Fungoides and Sézary Syndrome. 61
31045236 2019
50
CD8-positive lymphomatoid papulosis (type D): Some lesions may lack CD30 expression and overlap histologically with mycosis fungoides. 61
30520526 2019

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:

# Super pathways Score Top Affiliating Genes
1 11.6 TIA1 GZMB FAS
2 11.44 TYK2 TNFRSF8 GZMB FAS
3 11.3 TYK2 NPM1 ALK
4 10.9 NPM1 ALK
5 10.8 TYK2 TIA1

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 cellular response to mechanical stimulus GO:0071260 8.62 TNFRSF8 FAS

Sources for Lymphomatoid Papulosis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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