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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.
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.
Averages across everyone diagnosed, often years ago. A median is the middle of a group: half the people counted lived longer than the figure shown, and some lived far longer. Your stage, subtype, age, fitness and the treatment you receive matter more than the average, and the numbers are improving quickly.
Leukaemias, myeloma and MDS live in the marrow and blood; lymphomas grow in lymph nodes and spleen. The node stations are the disease map, not a route of spread, and staging counts them.
In lymphoma the node stations are the disease itself; staging (Ann Arbor / Lugano) counts how many regions and sides of the diaphragm are involved.
Same organ: High-grade B-cell lymphoma with MYC and BCL2 rearrangements (double-hit lymphoma), Mediastinal grey zone lymphoma, Primary effusion lymphoma, Plasmablastic lymphoma, T-cell/histiocyte-rich large B-cell lymphoma, EBV-positive diffuse large B-cell lymphoma, Primary large B-cell lymphoma of the testis, Gastric MALT lymphoma, Ocular adnexal MALT lymphoma, Extranodal NK/T-cell lymphoma, Adult T-cell leukaemia/lymphoma, ALK-positive anaplastic large cell lymphoma, ALK-negative anaplastic large cell lymphoma, Breast implant-associated anaplastic large cell lymphoma, Primary cutaneous anaplastic large cell lymphoma, Mycosis fungoides, Enteropathy-associated T-cell lymphoma, Monomorphic epitheliotropic intestinal T-cell lymphoma, Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma), Splenic marginal zone lymphoma, Nodal marginal zone lymphoma, Primary cutaneous marginal zone lymphoma, Primary cutaneous follicle centre lymphoma, Sezary syndrome, Nodal T-follicular helper cell lymphoma, angioimmunoblastic type (angioimmunoblastic T-cell lymphoma), Hepatosplenic T-cell lymphoma, Intravascular large B-cell lymphoma, Lymphomatoid granulomatosis, T-cell prolymphocytic leukaemia, Splenic B-cell lymphoma/leukaemia with prominent nucleoli (formerly B-cell prolymphocytic leukaemia and hairy cell leukaemia variant), T-cell large granular lymphocytic leukaemia, Mixed-phenotype acute leukaemia, Myeloid leukaemia of Down syndrome, Burkitt leukaemia, Marginal zone lymphoma, Cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome), Primary mediastinal (thymic) large B-cell lymphoma, Leukaemia (all types), Acute myeloid leukaemia, Acute lymphoblastic leukaemia, Chronic lymphocytic leukaemia, Chronic myeloid leukaemia (CML), Diffuse large B-cell lymphoma, Follicular lymphoma, Hodgkin lymphoma, Mantle cell lymphoma, Multiple myeloma, Non-Hodgkin lymphoma (all types), Myelodysplastic syndromes / neoplasms (MDS), Myeloproliferative neoplasms (PV, ET, myelofibrosis), Polycythaemia vera (PV), Essential thrombocythaemia (ET), Waldenström macroglobulinaemia, Hairy cell leukaemia, Peripheral T-cell lymphomas (including cutaneous T-cell lymphoma), Blastic plasmacytoid dendritic cell neoplasm (BPDCN), Burkitt lymphoma, HIV-associated (AIDS-related) lymphomas, Chronic myelomonocytic leukaemia and MDS/MPN overlap neoplasms, Systemic mastocytosis, Erdheim-Chester disease, Rosai-Dorfman disease and other histiocytic neoplasms, Langerhans cell histiocytosis (LCH), Post-transplant lymphoproliferative disorder (PTLD), FLT3-mutated acute myeloid leukaemia, IDH1- and IDH2-mutated acute myeloid leukaemia, NPM1-mutated and KMT2A-rearranged acute myeloid leukaemia, Secondary and therapy-related acute myeloid leukaemia, Acute promyelocytic leukaemia, Acute myeloid leukaemia in older or unfit patients, Smouldering multiple myeloma, Newly diagnosed multiple myeloma, transplant-eligible, Newly diagnosed multiple myeloma, transplant-ineligible, Relapsed or refractory multiple myeloma, Plasma cell leukaemia, Lower-risk myelodysplastic syndromes, Higher-risk myelodysplastic syndromes, Chronic lymphocytic leukaemia, first treatment, Relapsed or refractory chronic lymphocytic leukaemia, Richter transformation of chronic lymphocytic leukaemia, Chronic myeloid leukaemia, chronic phase, Chronic myeloid leukaemia, accelerated and blast phase, Primary myelofibrosis, Standard-risk B-cell acute lymphoblastic leukaemia in children, High-risk acute lymphoblastic leukaemia in children (high-risk B-ALL and T-ALL), Philadelphia chromosome-positive acute lymphoblastic leukaemia in children (Ph-positive ALL), Philadelphia chromosome-like acute lymphoblastic leukaemia (Ph-like or BCR::ABL1-like ALL), Infant acute lymphoblastic leukaemia (KMT2A-rearranged, under one year), Relapsed and refractory acute lymphoblastic leukaemia in children, Acute myeloid leukaemia in children, Erdheim-Chester disease, Rosai-Dorfman-Destombes disease, Single-system Langerhans cell histiocytosis (bone, skin or one other organ), Multisystem Langerhans cell histiocytosis (with or without risk-organ involvement), Indolent and smouldering systemic mastocytosis, Advanced systemic mastocytosis (aggressive SM, SM with an associated haematological neoplasm, mast cell leukaemia), Early-stage classical Hodgkin lymphoma (stage I to II), Advanced-stage classical Hodgkin lymphoma (stage III to IV), Nodular lymphocyte-predominant Hodgkin lymphoma (nodular lymphocyte-predominant B-cell lymphoma), Relapsed and refractory classical Hodgkin lymphoma
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.
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.
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.
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Query for this cancer: (TITLE:"Lymphomatoid papulosis" OR ABSTRACT:"Lymphomatoid papulosis" OR TITLE:"LyP" OR ABSTRACT:"LyP" OR TITLE:"Primary cutaneous CD30-positive T-cell lymphoproliferative disorder: lymphomatoid papulosis" OR ABSTRACT:"Primary cutaneous CD30-positive T-cell lymphoproliferative disorder: lymphomatoid papulosis" OR TITLE:"Lymphomatoid papulosis type A" OR ABSTRACT:"Lymphomatoid papulosis type A" OR TITLE:"Mucha-Habermann disease" OR ABSTRACT:"Mucha-Habermann disease") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Lymphomatoid papulosis, not a curated reading list.
Macaulay described a self-healing eruption whose biopsy looked malignant and whose course did not, and called it lymphomatoid papulosis.
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.
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.
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Bleeding that does not stop by itself, bleeding from more than one site, or new bruising in several places or one large area.
High-dose methotrexate requires normal renal function, hydration, urine alkalinisation and leucovorin rescue with level monitoring.
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