{"entity":{"id":"lymphomatoid-papulosis","kind":"cancer","name":"Lymphomatoid papulosis","aka":["LyP","Primary cutaneous CD30-positive T-cell lymphoproliferative disorder: lymphomatoid papulosis","Lymphomatoid papulosis type A","Mucha-Habermann disease"],"tldr":"A skin condition that keeps producing crops of small red bumps which ulcerate, crust and heal on their own over a few weeks, leaving small scars, and then come back. The biopsy looks like an aggressive lymphoma and the disease behaves nothing like one: nobody in the published series has died of it, but it carries a raised risk of a second lymphoma.","summary":"What it is. A chronic, relapsing condition of the skin in which crops of papules and small nodules appear, sometimes dozens at a time, go through a cycle of ulceration and crusting over three to twelve weeks, and heal on their own, often leaving a small scar. New crops follow. It can go on for years or decades, and it can stop.\n\nThe gap between the biopsy and the person. Under the microscope the lesions contain large, atypical CD30-positive cells that look like those of an aggressive lymphoma, and a pathologist who is given the slide without the history can reasonably report anaplastic large cell lymphoma. The diagnosis is made by putting the two together: lesions that come and go in crops and heal spontaneously, with that biopsy, are lymphomatoid papulosis. This is the clearest example in the lymphoma family of a diagnosis that cannot be made on the biopsy alone, and WHO-HAEM5 says as much of the skin lymphomas generally, that dermatological examination and clinical photographs are indispensable.\n\nWhere it sits in the classification. WHO-HAEM5 lists it among the primary cutaneous T-cell lymphomas as one of the two primary cutaneous CD30-positive T-cell lymphoproliferative disorders, the other being primary cutaneous anaplastic large cell lymphoma. The two are ends of one spectrum: the same person may have both, and the same T-cell clone can be found in both. Several histological types of lymphomatoid papulosis are described, named by letters, and they do not change the treatment or the outlook.\n\nThe risk that justifies follow-up. People with lymphomatoid papulosis have a raised risk of developing a second lymphoma, most often mycosis fungoides, primary cutaneous anaplastic large cell lymphoma or Hodgkin lymphoma, which may come before, with or after the skin lesions. That is the reason for continuing dermatological follow-up in a condition that is otherwise harmless, and it is the reason a new lump that behaves differently from the usual crops is biopsied.\n\nHow it is treated, which is often not at all. No treatment has been shown to prevent the second lymphoma or to change the course, so the aim is to control the lesions that bother the person. Observation with emollients and reassurance is a legitimate plan for somebody with a few lesions. Low-dose weekly methotrexate, phototherapy and potent topical steroids are used where the crops are frequent, numerous or scarring. Treatment suppresses the lesions and they return when it stops. Combination chemotherapy has no place.","asOf":"2026-09-29","wikipedia":"https://en.wikipedia.org/wiki/Lymphomatoid_papulosis","links":[{"label":"WHO Classification of Haematolymphoid Tumours, 5th edition: lymphoid neoplasms (Alaggio, Leukemia 2022)","url":"https://doi.org/10.1038/s41375-022-01620-2"},{"label":"International Consensus Classification of Mature Lymphoid Neoplasms (Campo, Blood 2022)","url":"https://doi.org/10.1182/blood.2022015851"},{"label":"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, Blood 2011)","url":"https://doi.org/10.1182/blood-2011-05-351346"},{"label":"CD30-positive cutaneous lymphoproliferative disorders: the Stanford experience in lymphomatoid papulosis and primary cutaneous anaplastic large cell lymphoma, 56 patients (Liu, J Am Acad Dermatol 2003)","url":"https://doi.org/10.1016/S0190-9622(03)02484-8"},{"label":"Lymphoma incidence, survival and prevalence 2004 to 2014, subtype analyses from the UK Haematological Malignancy Research Network (Smith, Br J Cancer 2015)","url":"https://doi.org/10.1038/bjc.2015.94"}],"tags":["heme","lymphoma","subtype-page"],"related":["primary-cutaneous-anaplastic-large-cell-lymphoma","cutaneous-t-cell-lymphoma","mycosis-fungoides","hodgkin-lymphoma","sezary-syndrome"],"cancers":[],"sections":[],"technologies":["histopathology-ihc"],"targets":["cd30"],"drugs":["methotrexate"],"companies":[],"institutions":[],"pathways":[],"terms":["lymphoma-classification-2022","lymphoma-tx-skin-directed-therapy","lymphoma-indolent-versus-aggressive","lymphoma-nodal-versus-extranodal"],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"group":"haematologic","burden":"In the United Kingdom population series that reports lymphoma by subtype, the primary cutaneous CD30-positive lymphoproliferative disorders, which group this condition with primary cutaneous anaplastic large cell lymphoma, accounted for 37 of 5,796 lymphomas, a European age-standardised rate of 0.13 per 100,000 a year, a median age at diagnosis of 52.9 years and five-year relative survival of 88.3 per cent. In the Stanford series of 56 patients with CD30-positive skin disease, no patient with lymphomatoid papulosis died of the disease and overall survival was 92 per cent at five and ten years.","subtypes":["Type A, the commonest, with scattered large CD30-positive cells in a mixed inflammatory background","Type B, which resembles mycosis fungoides down the microscope","Type C, which resembles anaplastic large cell lymphoma","Other described types, which do not change the treatment or the outlook"],"biomarkers":["CD30-positive large atypical cells on the biopsy, which on their own would suggest an aggressive lymphoma","The clinical course, which is the diagnosis: crops of lesions that ulcerate and heal on their own over weeks","A clonal T-cell receptor rearrangement in many cases, which does not make it a cancer in the way it would elsewhere","Absence of ALK","Continuing surveillance for a second lymphoma, most often mycosis fungoides, primary cutaneous anaplastic large cell lymphoma or Hodgkin lymphoma"],"standardOfCare":[{"setting":"Making the diagnosis, which needs the history as much as the biopsy","approach":"The biopsy shows large atypical CD30-positive cells that on their own would suggest an aggressive lymphoma. What makes the diagnosis is the course: crops of papules that ulcerate, crust and heal on their own over three to twelve weeks, often leaving small scars, recurring over years. Photographs and a dated history are part of the diagnostic record, not an extra. Staging confirms there is no disease outside the skin.","refs":["histopathology-ihc","cd30","lymphoma-tx-skin-directed-therapy","fdg-pet"],"guideline":{"version":"WHO Classification of Haematolymphoid Tumours, 5th edition (2022), with the International Consensus Classification (2022) where they differ","url":"https://doi.org/10.1038/s41375-022-01620-2"}},{"setting":"Treatment, which is often none","approach":"No treatment has been shown to change the course or to reduce the risk of a second lymphoma, so treatment is for the lesions that bother the person. Observation with emollients and an explanation is a legitimate plan for somebody with a few lesions, and in the published series nobody has died of this condition. Where crops are frequent, numerous or scarring, low-dose weekly methotrexate, phototherapy or potent topical steroids suppress them, and the lesions return when treatment stops. Combination chemotherapy has no place.","refs":["methotrexate","lymphoma-tx-skin-directed-therapy","lymphoma-tx-watch-and-wait"],"guideline":{"version":"EORTC, ISCL and USCLC consensus recommendations for primary cutaneous CD30-positive lymphoproliferative disorders (Blood 2011)","url":"https://doi.org/10.1182/blood-2011-05-351346"}},{"setting":"Why follow-up continues in a condition that does not shorten life","approach":"People with lymphomatoid papulosis have a raised risk of a second lymphoma, most often mycosis fungoides, primary cutaneous anaplastic large cell lymphoma or Hodgkin lymphoma, which may come before, alongside or after the skin lesions. Continuing dermatological review, and biopsy of any lump that behaves differently from the usual crops, is the reason for follow-up. Nothing prevents the second lymphoma, so the aim is to find it early.","refs":["mycosis-fungoides","primary-cutaneous-anaplastic-large-cell-lymphoma","hodgkin-lymphoma","histopathology-ihc"],"guideline":{"version":"NCI PDQ: adult non-Hodgkin lymphoma treatment","url":"https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq"}}],"stateOfArt":[],"history":[{"year":1968,"title":"Named","note":"Macaulay described a self-healing eruption whose biopsy looked malignant and whose course did not, and called it lymphomatoid papulosis.","refs":[]},{"year":2003,"title":"No deaths from the disease in a single-centre series","note":"In the Stanford series of CD30-positive skin lymphoproliferative disorders, no patient with lymphomatoid papulosis died of the disease, and overall survival was 92 per cent at five and ten years.","refs":[]},{"year":2011,"title":"International consensus recommendations","note":"The EORTC, International Society for Cutaneous Lymphomas and United States Cutaneous Lymphoma Consortium panel set out definitions, endpoints and treatment recommendations, and recorded that the level of evidence for most treatments is low.","refs":[]}],"pipeline":[],"openProblems":["No treatment has been shown to reduce the risk of a second lymphoma, so treatment is for symptoms only and over-treatment is a real harm in a condition that does not shorten life.","There is no way to predict which patient will develop a second lymphoma, so everybody is followed up indefinitely.","The consensus recommendations state that the evidence behind nearly every treatment is retrospective and small."],"parent":"cutaneous-t-cell-lymphoma"},"route":"/cancers/lymphomatoid-papulosis/","neighbours":{"cancer":[{"id":"cutaneous-t-cell-lymphoma","kind":"cancer","name":"Cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome)","route":"/cancers/cutaneous-t-cell-lymphoma/"},{"id":"hodgkin-lymphoma","kind":"cancer","name":"Hodgkin lymphoma","route":"/cancers/hodgkin-lymphoma/"},{"id":"mycosis-fungoides","kind":"cancer","name":"Mycosis fungoides","route":"/cancers/mycosis-fungoides/"},{"id":"primary-cutaneous-anaplastic-large-cell-lymphoma","kind":"cancer","name":"Primary cutaneous anaplastic large cell lymphoma","route":"/cancers/primary-cutaneous-anaplastic-large-cell-lymphoma/"},{"id":"sezary-syndrome","kind":"cancer","name":"Sezary syndrome","route":"/cancers/sezary-syndrome/"}],"technology":[{"id":"fdg-pet","kind":"technology","name":"FDG PET","route":"/technologies/fdg-pet/"},{"id":"histopathology-ihc","kind":"technology","name":"Histopathology & immunohistochemistry","route":"/technologies/histopathology-ihc/"}],"target":[{"id":"cd30","kind":"target","name":"CD30","route":"/targets/cd30/"}],"drug":[{"id":"methotrexate","kind":"drug","name":"Methotrexate","route":"/drugs/methotrexate/"}],"term":[{"id":"lymphoma-indolent-versus-aggressive","kind":"term","name":"Indolent and aggressive lymphoma","route":"/terms/lymphoma-indolent-versus-aggressive/"},{"id":"lymphoma-nodal-versus-extranodal","kind":"term","name":"Nodal and extranodal lymphoma","route":"/terms/lymphoma-nodal-versus-extranodal/"},{"id":"lymphoma-tx-skin-directed-therapy","kind":"term","name":"Skin-directed therapy in mycosis fungoides: creams, light and small radiotherapy fields","route":"/terms/lymphoma-tx-skin-directed-therapy/"},{"id":"lymphoma-classification-2022","kind":"term","name":"The two lymphoma classifications of 2022 (WHO-HAEM5 and ICC)","route":"/terms/lymphoma-classification-2022/"},{"id":"lymphoma-tx-watch-and-wait","kind":"term","name":"Watch and wait in lymphoma: when the right treatment is none yet","route":"/terms/lymphoma-tx-watch-and-wait/"}]}}