# Lymphomatoid papulosis

Source: https://onco.cc/cancers/lymphomatoid-papulosis/  
OnCo record `lymphomatoid-papulosis` (Cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

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.

The 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.

Where 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.

The 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.

How 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.

## Fields

- Kind: Cancer
- Last checked: 2026-09-29
- Also known as: LyP; Primary cutaneous CD30-positive T-cell lymphoproliferative disorder: lymphomatoid papulosis; Lymphomatoid papulosis type A; Mucha-Habermann disease
- Tags: heme; lymphoma; subtype-page
- 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

## Sections of this record

The page is a hub with ten sections in reading order; large sections have their own page. The same plan as JSON: https://onco.cc/api/v1/cancers/lymphomatoid-papulosis/sections.json

- Overview (on the hub): The TL;DR, the family this cancer belongs to, the organ, who gets it and what the state of the art is. https://onco.cc/cancers/lymphomatoid-papulosis/#overview
- Types and stages (on the hub): Anatomy, the subtypes and how they differ, how it is staged, and where advanced disease spreads. https://onco.cc/cancers/lymphomatoid-papulosis/#what-it-is [4 subtypes]
- Symptoms and diagnosis (on the hub): How this cancer shows itself, how the diagnosis is confirmed, and the biomarkers clinicians test for. https://onco.cc/cancers/lymphomatoid-papulosis/#finding-it [5 biomarkers]
- Treatment (on the hub): The standard of care by setting, the medicines, surgery and radiotherapy named in it, and the regimens behind them. https://onco.cc/cancers/lymphomatoid-papulosis/#treating-it [3 settings, 1 decision with options]
- Trials and papers (on the hub): Trials recruiting now, the landmark trials, the trials held by this cancer's subtypes, the key papers and what they mean, the latest literature, and the milestones year by year. https://onco.cc/cancers/lymphomatoid-papulosis/#evidence [3 milestones]
- Biology and targets (on the hub): The molecular landscape: the targets and how often each appears, the pathways, the mechanics stages and the preclinical models. https://onco.cc/cancers/lymphomatoid-papulosis/#science [1 target]
- Countries and centres (own page): Cases by country, the UK and NHS pathway and other country lenses, the expert centres with trials on record, and the centres named on this cancer's subtypes. https://onco.cc/cancers/lymphomatoid-papulosis/where-you-are/
- Decisions and support (on the hub): The decisions you may face, the aids that walk through them, the warnings on record, the first sixty days and the questions to ask. https://onco.cc/cancers/lymphomatoid-papulosis/#living-with-it [12 questions, 2 red cards]
- Pipeline and open problems (own page): Everything in development, the medicines held by this cancer's subtypes, the open problems and what is being done about them, the roadmaps, and what changed on this record. https://onco.cc/cancers/lymphomatoid-papulosis/coming/ [1 medicine, 3 open problems]
- Data (own page): Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked. https://onco.cc/cancers/lymphomatoid-papulosis/data/ [10 connected records]

## Standard of care

- Making the diagnosis, which needs the history as much as the biopsy: 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. ([Histopathology & immunohistochemistry](https://onco.cc/technologies/histopathology-ihc/), [CD30](https://onco.cc/targets/cd30/), [Skin-directed therapy in mycosis fungoides: creams, light and small radiotherapy fields](https://onco.cc/terms/lymphoma-tx-skin-directed-therapy/), [FDG PET](https://onco.cc/technologies/fdg-pet/))
- Treatment, which is often none: 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. ([Methotrexate](https://onco.cc/drugs/methotrexate/), [Skin-directed therapy in mycosis fungoides: creams, light and small radiotherapy fields](https://onco.cc/terms/lymphoma-tx-skin-directed-therapy/), [Watch and wait in lymphoma: when the right treatment is none yet](https://onco.cc/terms/lymphoma-tx-watch-and-wait/))
- Why follow-up continues in a condition that does not shorten life: 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. ([Mycosis fungoides](https://onco.cc/cancers/mycosis-fungoides/), [Primary cutaneous anaplastic large cell lymphoma](https://onco.cc/cancers/primary-cutaneous-anaplastic-large-cell-lymphoma/), [Hodgkin lymphoma](https://onco.cc/cancers/hodgkin-lymphoma/), [Histopathology & immunohistochemistry](https://onco.cc/technologies/histopathology-ihc/))

## Open problems

- 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.

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Lymphomatoid_papulosis
- WHO Classification of Haematolymphoid Tumours, 5th edition: lymphoid neoplasms (Alaggio, Leukemia 2022): https://doi.org/10.1038/s41375-022-01620-2
- International Consensus Classification of Mature Lymphoid Neoplasms (Campo, Blood 2022): https://doi.org/10.1182/blood.2022015851
- 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): https://doi.org/10.1182/blood-2011-05-351346
- 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): https://doi.org/10.1016/S0190-9622(03)02484-8
- Lymphoma incidence, survival and prevalence 2004 to 2014, subtype analyses from the UK Haematological Malignancy Research Network (Smith, Br J Cancer 2015): https://doi.org/10.1038/bjc.2015.94

## Connected records

- cancers: [Cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome)](https://onco.cc/cancers/cutaneous-t-cell-lymphoma/), [Hodgkin lymphoma](https://onco.cc/cancers/hodgkin-lymphoma/), [Mycosis fungoides](https://onco.cc/cancers/mycosis-fungoides/), [Primary cutaneous anaplastic large cell lymphoma](https://onco.cc/cancers/primary-cutaneous-anaplastic-large-cell-lymphoma/), [Sezary syndrome](https://onco.cc/cancers/sezary-syndrome/)
- technologies: [FDG PET](https://onco.cc/technologies/fdg-pet/), [Histopathology & immunohistochemistry](https://onco.cc/technologies/histopathology-ihc/)
- targets: [CD30](https://onco.cc/targets/cd30/)
- drugs: [Methotrexate](https://onco.cc/drugs/methotrexate/)
- terms: [Indolent and aggressive lymphoma](https://onco.cc/terms/lymphoma-indolent-versus-aggressive/), [Nodal and extranodal lymphoma](https://onco.cc/terms/lymphoma-nodal-versus-extranodal/), [Skin-directed therapy in mycosis fungoides: creams, light and small radiotherapy fields](https://onco.cc/terms/lymphoma-tx-skin-directed-therapy/), [The two lymphoma classifications of 2022 (WHO-HAEM5 and ICC)](https://onco.cc/terms/lymphoma-classification-2022/), [Watch and wait in lymphoma: when the right treatment is none yet](https://onco.cc/terms/lymphoma-tx-watch-and-wait/)

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JSON: https://onco.cc/api/v1/entities/lymphomatoid-papulosis.json