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Lymphomatoid papulosis

MedGen UID:: 61534
•Concept ID:: C0206182
•: Neoplastic Process

Synonym:	LyP
SNOMED CT:	Lymphomatoid papulosis (397353001); Lymphomatoid papulosis (31047003)

Monarch Initiative:	MONDO:0020326
Orphanet:	ORPHA98842

Definition

Lymphomatoid papulosis (LyP) is a rare cutaneous condition characterized by chronic, recurrent, and self-regressing papulonodular skin eruptions. It belongs to the spectrum of primary cutaneous CD30+ lymphoproliferative disorders, along with primary cutaneous anaplastic large cell lymphoma (primary C-ALCL; see this term) with which it shares overlapping clinical and histopathologic features. [from ORDO]

Term Hierarchy

GTR
MeSH
Orphanet

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVLymphomatoid papulosis

Disorder by Site
- Hematopoietic and Lymphoid System Disorder
  - Hematologic disorder
    - Hematopoietic and lymphoid cell neoplasm
      - Cutaneous Hematopoietic and Lymphoid Cell Neoplasm
        Cutaneous Mature T-Cell and NK-Cell Neoplasm
        Lymphomatoid papulosis
        Lymphomatoid Papulosis with DUSP22-IRF4 Gene Rearrangement
        Type A Lymphomatoid Papulosis
        Type B Lymphomatoid Papulosis
        Type C Lymphomatoid Papulosis
        Type D Lymphomatoid Papulosis
        Type E Lymphomatoid Papulosis

Follow this link to review classifications for Lymphomatoid papulosis in Orphanet.

Professional guidelines

PubMed

A Practical Guide to the Diagnosis, Evaluation, and Treatment of Cutaneous T-Cell Lymphoma.

Shimshak S, Sokumbi O, Isaq N, Goyal A, Comfere N
Dermatol Clin 2023 Jan;41(1):209-229. doi: 10.1016/j.det.2022.07.019. PMID: 36410979

Diagnosis and Management of Cutaneous Lymphomas Including Cutaneous T-cell Lymphoma.

Zic JA
Med Clin North Am 2021 Jul;105(4):737-755. doi: 10.1016/j.mcna.2021.04.010. PMID: 34059248

Practical Management of CD30⁺ Lymphoproliferative Disorders.

Hughey LC
Dermatol Clin 2015 Oct;33(4):819-33. Epub 2015 Aug 29 doi: 10.1016/j.det.2015.05.013. PMID: 26433852

See all (34)

Recent clinical studies

Etiology

Cutaneous T-cell Lymphoma.

Weiner DM, Rook AH
Hematol Oncol Clin North Am 2024 Oct;38(5):1087-1110. Epub 2024 Jul 29 doi: 10.1016/j.hoc.2024.05.012. PMID: 39079789

Primary cutaneous T-cell lymphomas other than mycosis fungoides and Sézary syndrome. Part I: Clinical and histologic features and diagnosis.

Stoll JR, Willner J, Oh Y, Pulitzer M, Moskowitz A, Horwitz S, Myskowski P, Noor SJ
J Am Acad Dermatol 2021 Nov;85(5):1073-1090. Epub 2021 Apr 30 doi: 10.1016/j.jaad.2021.04.080. PMID: 33940098

Lymphomatoid papulosis: an update and review.

Martinez-Cabriales SA, Walsh S, Sade S, Shear NH
J Eur Acad Dermatol Venereol 2020 Jan;34(1):59-73. Epub 2019 Oct 14 doi: 10.1111/jdv.15931. PMID: 31494989

The 2018 update of the WHO-EORTC classification for primary cutaneous lymphomas.

Willemze R, Cerroni L, Kempf W, Berti E, Facchetti F, Swerdlow SH, Jaffe ES
Blood 2019 Apr 18;133(16):1703-1714. Epub 2019 Jan 11 doi: 10.1182/blood-2018-11-881268. PMID: 30635287 Free PMC Article

Cutaneous T-cell Lymphoma.

Pulitzer M
Clin Lab Med 2017 Sep;37(3):527-546. doi: 10.1016/j.cll.2017.06.006. PMID: 28802499 Free PMC Article

See all (218)

Diagnosis

Primary cutaneous T-cell lymphomas other than mycosis fungoides and Sézary syndrome. Part I: Clinical and histologic features and diagnosis.

Stoll JR, Willner J, Oh Y, Pulitzer M, Moskowitz A, Horwitz S, Myskowski P, Noor SJ
J Am Acad Dermatol 2021 Nov;85(5):1073-1090. Epub 2021 Apr 30 doi: 10.1016/j.jaad.2021.04.080. PMID: 33940098

Lymphomatoid papulosis.

Sica A, Vitiello P, Sorriento A, Ronchi A, Calogero A, Sagnelli C, Troiani T, Fasano M, Dodaro CA, Franco R, Casale B, Santangelo M, Ciccozzi M, Ciardiello F, Argenziano G, Moscarella E
Minerva Med 2020 Apr;111(2):166-172. Epub 2020 Jan 20 doi: 10.23736/S0026-4806.19.06395-X. PMID: 31958921

Lymphomatoid papulosis: an update and review.

Martinez-Cabriales SA, Walsh S, Sade S, Shear NH
J Eur Acad Dermatol Venereol 2020 Jan;34(1):59-73. Epub 2019 Oct 14 doi: 10.1111/jdv.15931. PMID: 31494989

The 2018 update of the WHO-EORTC classification for primary cutaneous lymphomas.

Willemze R, Cerroni L, Kempf W, Berti E, Facchetti F, Swerdlow SH, Jaffe ES
Blood 2019 Apr 18;133(16):1703-1714. Epub 2019 Jan 11 doi: 10.1182/blood-2018-11-881268. PMID: 30635287 Free PMC Article

Cutaneous T-cell Lymphoma.

Pulitzer M
Clin Lab Med 2017 Sep;37(3):527-546. doi: 10.1016/j.cll.2017.06.006. PMID: 28802499 Free PMC Article

See all (416)

Therapy

Histopathological Markers for Target Therapies in Primary Cutaneous Lymphomas.

Sonego B, Ibatici A, Rivoli G, Angelucci E, Sola S, Massone C
Cells 2023 Nov 20;12(22) doi: 10.3390/cells12222656. PMID: 37998391 Free PMC Article

Lymphomatoid Papulosis and Other Lymphoma-Like Diseases.

Moy A, Sun J, Ma S, Seminario-Vidal L
Dermatol Clin 2019 Oct;37(4):471-482. Epub 2019 Aug 6 doi: 10.1016/j.det.2019.05.005. PMID: 31466587

Antibody-Based Therapies for Cutaneous T-Cell Lymphoma.

Welborn M, Duvic M
Am J Clin Dermatol 2019 Feb;20(1):115-122. doi: 10.1007/s40257-018-0402-5. PMID: 30430444

Lymphomatoid papulosis.

Wiznia LE, Cohen JM, Beasley JM, Meehan SA, Latkowski JM
Dermatol Online J 2018 Dec 15;24(12) PMID: 30677799

Phototherapy of cutaneous T-cell lymphomas.

Trautinger F
Photochem Photobiol Sci 2018 Dec 5;17(12):1904-1912. doi: 10.1039/c8pp00170g. PMID: 30325389

See all (133)

Prognosis

Primary cutaneous T-cell lymphomas other than mycosis fungoides and Sézary syndrome. Part I: Clinical and histologic features and diagnosis.

Stoll JR, Willner J, Oh Y, Pulitzer M, Moskowitz A, Horwitz S, Myskowski P, Noor SJ
J Am Acad Dermatol 2021 Nov;85(5):1073-1090. Epub 2021 Apr 30 doi: 10.1016/j.jaad.2021.04.080. PMID: 33940098

Lymphomatoid papulosis.

Wagner G, Rose C, Klapper W, Sachse MM
J Dtsch Dermatol Ges 2020 Mar;18(3):199-205. Epub 2020 Feb 26 doi: 10.1111/ddg.14041. PMID: 32100965

Lymphomatoid papulosis.

Lymphomatoid papulosis: an update and review.

Martinez-Cabriales SA, Walsh S, Sade S, Shear NH
J Eur Acad Dermatol Venereol 2020 Jan;34(1):59-73. Epub 2019 Oct 14 doi: 10.1111/jdv.15931. PMID: 31494989

The 2018 update of the WHO-EORTC classification for primary cutaneous lymphomas.

Willemze R, Cerroni L, Kempf W, Berti E, Facchetti F, Swerdlow SH, Jaffe ES
Blood 2019 Apr 18;133(16):1703-1714. Epub 2019 Jan 11 doi: 10.1182/blood-2018-11-881268. PMID: 30635287 Free PMC Article

See all (287)

Clinical prediction guides

Lymphomatoid papulosis in children and adolescents: A clinical and histopathologic retrospective cohort.

Georgesen C, Magro C
Ann Diagn Pathol 2020 Jun;46:151486. Epub 2020 Mar 3 doi: 10.1016/j.anndiagpath.2020.151486. PMID: 32172217

Lymphomatoid Papulosis and Other Lymphoma-Like Diseases.

Moy A, Sun J, Ma S, Seminario-Vidal L
Dermatol Clin 2019 Oct;37(4):471-482. Epub 2019 Aug 6 doi: 10.1016/j.det.2019.05.005. PMID: 31466587

CD30+ T-cell lymphoproliferative disorders.

Martin JM, Wu H, Barta SK
Chin Clin Oncol 2019 Feb;8(1):4. Epub 2018 Oct 11 doi: 10.21037/cco.2018.09.06. PMID: 30525751

Aggressive cutaneous T-cell lymphomas.

Junkins-Hopkins Md JM
Semin Diagn Pathol 2017 Jan;34(1):44-59. Epub 2016 Dec 24 doi: 10.1053/j.semdp.2016.11.004. PMID: 28187925

Lymphomatoid papulosis.

Willemze R
Dermatol Clin 1985 Oct;3(4):735-47. PMID: 3916178

See all (143)

Recent systematic reviews

Pediatric cellular neurothekeoma: Seven cases and systematic review of the literature.

Murphrey M, Huy Nguyen A, White KP, Krol A, Bernert R, Yarbrough K
Pediatr Dermatol 2020 Mar;37(2):320-325. Epub 2020 Jan 12 doi: 10.1111/pde.14043. PMID: 31930561

Lymphomatoid Papulosis in Children and Adolescents: A Systematic Review.

Wieser I, Wohlmuth C, Nunez CA, Duvic M
Am J Clin Dermatol 2016 Aug;17(4):319-27. doi: 10.1007/s40257-016-0192-6. PMID: 27138554

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.

Kempf W, Pfaltz K, Vermeer MH, Cozzio A, Ortiz-Romero PL, Bagot M, Olsen E, Kim YH, Dummer R, Pimpinelli N, Whittaker S, Hodak E, Cerroni L, Berti E, Horwitz S, Prince HM, Guitart J, Estrach T, Sanches JA, Duvic M, Ranki A, Dreno B, Ostheeren-Michaelis S, Knobler R, Wood G, Willemze R
Blood 2011 Oct 13;118(15):4024-35. Epub 2011 Aug 12 doi: 10.1182/blood-2011-05-351346. PMID: 21841159 Free PMC Article

See all (3)

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Lymphomatoid papulosis

Definition

Term Hierarchy

Professional guidelines

PubMed

Recent clinical studies

Etiology

Diagnosis

Therapy

Prognosis

Clinical prediction guides

Recent systematic reviews

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Practice guidelines

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