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Lymphomatoid Papulosis

Last updated Nov. 3, 2018

Approved by: Maulik P. Purohit MD, MPH

Lymphomatoid Papulosis (LyP) is a rare cutaneous T-cell non-Hodgkin’s lymphoma. It is considered to be CD-30 positive T-cell lymphoproliferative disorder (disorder causing excess production of lymphocytes).


What are the other Names for this Condition? (Also known as/Synonyms)

  • LyP (Lymphomatoid Papulosis)

What is Lymphomatoid Papulosis? (Definition/Background Information)

  • Lymphomatoid Papulosis (LyP) is a rare cutaneous T-cell non-Hodgkin’s lymphoma. It is considered to be CD-30 positive T-cell lymphoproliferative disorder (disorder causing excess production of lymphocytes). The condition may be typically seen from childhood to middle-age
  • This is a type of primary cutaneous non-Hodgkin lymphoma, which means that it is a lymphoma that originates first in the skin. It can later involve other parts of the body including the lymph nodes and bone marrow. This is known as systemic Lymphomatoid Papulosis
  • The condition is normally diagnosed using a biopsy of the involved skin or using a biopsy of the swollen lymph nodes or affected organ. However, various blood tests and imaging studies may also be performed to help in the diagnosis
  • There is no cure for the condition. The treatment of Lymphomatoid Papulosis mostly involves skin therapy; typically systemic therapy is not necessary. In a majority of individuals LyP is self-limiting, though it may chronically appear and disappear over time
  • The prognosis depends on many factors including the progression of the condition, response to treatment, and overall health of the individual. In general, the prognosis is good with appropriate treatment, since Lymphomatoid Papulosis is a low-grade malignancy

General information on lymphoma and lymphocytes:

  • Lymphoma is a type of cancer stemming from uncontrollably dividing lymphocytes (type of white blood cells). There are two types of lymphomas:
    • Hodgkin lymphoma
    • Non-Hodgkin lymphoma
  • Lymphocytes are the main white blood cells found in the lymph, which is the fluid of the lymphatic system; just as blood is the fluid of the circulatory system
  • Lymphocytes are made in bone marrow, and can develop into either B-cells or T-cells. T-cells mature in the thymus; Lymphomatoid Papulosis arises from cancerous T-cells
  • Lymph results from filtration of blood as it travels to and from tissues. Lymph is colorless because it lacks red blood cells; instead, it contains lymphocytes. It is central to the immune system
  • There are 3 different kinds of lymphocytes:
    • T-lymphocytes or T cells: They help combat infections and abnormalities within the cells (cell-mediated immunity). They fight viruses and cancerous cells
    • B-lymphocytes or B cells: They produce antibodies that are bodily defense proteins, which target foreign invaders outside the cells (humoral immunity). They fight bacterial cells, cell fragments, and other immunogenic elements
    • Natural killer cells or NK cells: They perform diverse functions related to both cell-mediated and humoral immunity. They also scout for cancer cells, a process called immune surveillance

Who gets Lymphomatoid Papulosis? (Age and Sex Distribution)

  • Lymphomatoid Papulosis is a rare disorder with an incidence of 1 per million population
  • The condition generally affects adults in the age group 30-80 years. Even children may be affected
  • Both males and females can be affected. Some studies show an equal degree of incidence between them, while some others show that males are affected more than females in a 3:2 ratio
  • All races and ethnic groups can be affected; however, Caucasians are affected more than other racial groups

What are the Risk Factors for Lymphomatoid Papulosis? (Predisposing Factors)

The risk factors for Lymphomatoid Papulosis are generally unknown. However, in about 10% of the cases, the condition is associated with the following disorders:

  • Mycoses fungoides (MF)
  • Hodgkin’s lymphoma
  • Anaplastic large cell lymphoma (ALCL)

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Lymphomatoid Papulosis? (Etiology)

Lymphocytes are a type of white blood cells that are responsible for providing immunity in the human body. B-cells and T-cells are the two different types of lymphocytes. When under certain circumstances, the lymphocytes grow and multiply abnormally, it leads to a condition called as lymphoma, which is a most common type of cancer. There are 2 types of lymphoma:

  • Hodgkin lymphoma
  • Non-Hodgkin lymphoma

The cause of Lymphomatoid Papulosis is unknown. There may be certain genetic defects, such as translocation, which is a juxtaposition of regions of the chromosomes. This may result in:

  • Change of regulatory elements of certain cancer-causing genes called as oncogenes, which can lead to increased production of their mRNA (overexpression), thus increasing their protein levels
  • Exchange of protein coding regions of gene, giving rise to new proteins that can stimulate the inappropriate growth of cells

It is believed that the abnormal development of lymphocytes gives rise to cancerous cells leading to the formation of this condition. Nevertheless, how this occurs and the factors that cause it remain under investigation.

Note: Lymphomatoid Papulosis is non-contagious and is not transmitted from one individual to another. One cannot contract the condition through close physical interaction with the affected individuals.

What are the Signs and Symptoms of Lymphomatoid Papulosis?

The signs and symptoms of Lymphomatoid Papulosis mostly involve the skin. These may include:

  • Presence of nodules and patches
  • Papules and nodules on skin that gets bigger and smaller over time
  • The skin lesions ‘come and go’ on their own; some stay for weeks-to-months and then recede
  • Sometimes, they can disappear leaving behind a scar
  • The skin lesions are usually less than 1 cm, but can be up to 3 cm in size
  • Any skin part on the body may be affected
  • But as they grow they can ulcerate and spread to other parts of the body

In rare cases, if other organs are involved, the following general signs and symptoms may be seen, which include:

  • Unintentional weight loss; changes in appetite
  • Fatigue and weakness, headache
  • High temperatures and excessive night sweats (may be recurrent)
  • Anemia (low red blood cell count)
  • Low lymphocyte count, established by a blood test
  • Frequent infections
  • Trouble breathing
  • Low blood pressure
  • Back pain
  • Swelling of the legs
  • Abdominal pain and swelling; constipation
  • Frequent urination

How is Lymphomatoid Papulosis Diagnosed?

Lymphomatoid Papulosis diagnosis is performed by obtaining skin biopsy samples from the affected region and examining them under a microscope to detect the cancerous cells. Other methods to aid in the study-diagnosis of LyP may include:

  • A thorough physical examination and a complete medical history, which is very important
  • Blood tests that may include:
    • Complete blood cell count (CBC) blood test
    • Absolute lymphocyte count on peripheral blood
    • Liver function blood test (LFT)
    • Lactate dehydrogenase (LDH) blood test
  • Skin biopsy from the affected area:
    • A biopsy of the tumor is performed and sent to a laboratory for a pathological examination. A pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis. Examination of the biopsy under a microscope by a pathologist is considered to be gold standard in arriving at a conclusive diagnosis
    • Biopsy specimens are studied initially using Hematoxylin and Eosin staining. The pathologist then decides on additional studies depending on the clinical situation
    • Sometimes, the pathologist may perform special studies, which may include immunohistochemical stains, molecular testing, flow cytometric analysis and very rarely, electron microscopic studies, to assist in the diagnosis
  • In rare cases of systemic involvement, other diagnostic tools may be used to study/analyze the condition

Note: Differential diagnoses, to eliminate other lymphoma types may be often considered, before arriving at a definitive diagnosis.

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Lymphomatoid Papulosis?

The complications due to Lymphomatoid Papulosis may include:

  • Very rarely, involvement to local and distant organs (the tumor can spread from the lymph nodes to other parts of the body)
  • Loss of function of the organ/area to which the cancer has spread
  • Immunosuppression leading to frequent infections
  • It is a chronic disorder that ‘comes and goes’
  • Individuals with Lymphomatoid Papulosis may have other types of lymphomas

How is Lymphomatoid Papulosis Treated?

There is no definitive cure for Lymphomatoid Papulosis and a treatment is undertaken to control or reduce the severity of the skin signs and symptoms. This may involve the following measures:

  • Sunlight therapy (or exposure to ultraviolet light)
  • Use of topical steroids and chemotherapy
  • Systemic chemotherapy
  • Radiation therapy to affected (local) skin area
  • Other systemic treatment measures, such as the use of interferons, retinoids, etc.
  • Biological therapy

Note: Systemic therapy is used only in rare case of a systemic involvement, when other body organs or systems are affected.

Your healthcare provider will determine the best course of treatment depending on your individual circumstances. Also, follow-up care with regular screening and check-ups are important post-treatment.

How can Lymphomatoid Papulosis be Prevented?

Currently, the cause of Lymphomatoid Papulosis is unknown; hence, there are no known methods to prevent the tumor occurrence. Nevertheless, considering the following factors may help in preventing cancers:

  • Healthy diet and exercise, as well as avoidance of unnecessary exposure to chemicals, may help decrease the risk of cancer
  • Avoiding or stopping smoking
  • Genetic testing in individuals with a family history of cancers
  • In order to avoid a relapse, or be prepared for a recurrence, the entire diagnosis, treatment process, drugs administered, etc. should be well-documented and follow-up measures initiated

Regular medical screening at periodic intervals with blood tests, scans, and physical examinations, are mandatory. Often several years of active vigilance are crucial and necessary.

What is the Prognosis of Lymphomatoid Papulosis? (Outcomes/Resolutions)

  • The prognosis of Lymphomatoid Papulosis in a majority of individuals is excellent, since LyP is a self-limiting condition that resolves on its own. On the other hand, the condition is chronic and may periodically appear and disappear
  • The prognosis depends upon a set of several factors, which include:
    • Stage of tumor: With lower-stage tumors, when the tumor is confined to site of origin, the prognosis is usually excellent with appropriate therapy. In higher-stage tumors, such as tumors with metastasis, the prognosis is poor
    • Overall health of the individual: Individuals with overall excellent health have better prognosis compared with those with poor health
    • Age of the individual: Older individuals generally have poorer prognosis than younger individuals
    • The size of the tumor: Individuals with small-sized tumors fare better than those with large-sized tumors
    • Individuals with bulky disease have a poorer prognosis
    • Involvement of vital organs may complicate the condition
    • The surgical respectability of the tumor (meaning, if the tumor can be removed completely) - it is a rare option
    • Whether the tumor is occurring for the first time, or is a recurrent tumor. Recurring tumors have worse prognosis compared to tumors that do not recur
    • Response to treatment: Tumors that respond to treatment have better prognosis compared to tumors that do not respond to treatment
    • Progression of the condition makes the outcome worse (progressive Lymphomatoid Papulosis)
  • An early diagnosis and prompt treatment of the tumor generally yields better outcomes than a late diagnosis and delayed treatment

Additional and Relevant Useful Information for Lymphomatoid Papulosis:

  • T-cell lymphomas are less common than B-cell lymphomas
  • Treatment for Lymphomatoid Papulosis can cause physical and emotional distress; supportive care and encouragement, help positively and can bring a measure of relief to the patients

The following article link will help you understand leukemia and lymphoma (blood cancer):

https://www.dovemed.com/diseases-conditions/leukemia-and-lymphoma/

What are some Useful Resources for Additional Information?


References and Information Sources used for the Article:


Helpful Peer-Reviewed Medical Articles:


Reviewed and Approved by a member of the DoveMed Editorial Board
First uploaded: Feb. 17, 2016
Last updated: Nov. 3, 2018