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There are many types of skin lymphomas. Classifying them can be confusing (even for many doctors) because many of them are not very common.
The main systems used to classify skin lymphoma are:
The two systems are very similar and are based mainly on:
Most skin lymphomas are T-cell lymphomas. These are sometimes called cutaneous T-cell lymphomas (CTCLs).
Nearly half of all skin lymphomas are mycosis fungoides (MF). MF can occur in people of any age, but most who get it are in their 50s or 60s. Men are almost twice as likely as women to develop this lymphoma.
The first sign of this disease is usually one or more dry, scaly, pink or red areas (called patches) on the skin that are often very itchy. Often these patches are the only symptom of MF.
In some people, MF might appear as thickened, raised areas on the skin with distinct borders (called plaques). Plaques most often start as patches, but in some people they might be the first sign of MF.
MF can also appear as tumors (nodules), which are typically larger and have a rounded, more distinct appearance. In some tumors, the skin might break open (ulcerate). Tumors might appear on their own, or they might develop from plaques over time.
Because MF can be confused with other skin problems, it can be hard to diagnose at first. Several biopsies of the areas might be needed before the diagnosis is confirmed.
Over time, MF can spread across the skin or invade lymph nodes and organs like the liver. In many people this disease grows slowly, but it can sometimes grow more quickly, especially in older people. Some people with MF may go on to develop Sezary syndrome (see below).
People with MF can also have weakened immune systems, which can increase their risk for infections.
Rare variants of MF include folliculotropic MF, pagetoid reticulosis, and granulomatous slack skin.
This is often thought of as an advanced form of mycosis fungoides (MF), but these are somewhat different diseases.
People with SS typically have a very itchy, scaly red rash that can look like a severe sunburn. This is called generalized erythroderma. The skin is often thickened. Lymphoma cells, called Sezary cells, can be found in the blood and the lymph nodes.
People with SS often have weakened immune systems, which increases their risk of serious infections.
This rare type of T-cell lymphoma is more likely to start in other parts of the body, but it can sometimes be confined to the skin.
ATLL is linked to infection with the HTLV-1 virus (although most people infected with this virus do not get lymphoma). It is much more common in Japan and the Caribbean islands than in other parts of the world.
This lymphoma often grows quickly, but in some cases it advances slowly or even shrinks on its own for a time.
This lymphoma usually starts as one or a few tumors on the skin. The tumors can vary in size, and some may break open (ulcerate).
Most people with this disease are in their 50s or 60s, but it can also happen in children. It is found at least twice as often in men as in women. In most cases, it doesn’t spread beyond the skin and the prognosis (outlook) is very good.
Lymphomatoid papulosis is a benign, slow-growing disease that often comes and goes on its own, even without treatment. It often begins as several large pimple-like lesions that may break open in the middle.
This disorder is seen in younger people more than other T-cell skin lymphomas, with an average age of around 45. Men get this disease more often than women.
Lymphomatoid papulosis doesn’t spread to internal organs and is not fatal. Treatment often isn't needed right away, especially if it isn’t causing any bothersome symptoms.
Rarely, some people with this skin disorder develop another, more serious type of lymphoma.
This rare lymphoma invades the deepest layers of the skin, where it causes nodules (lumps) to form. Most often these are on the legs, but they can happen anywhere on the body.
This lymphoma affects all ages and is slightly more common in females. It usually grows slowly and tends to have a good outlook.
This rare type of lymphoma can start in T cells or in other lymphocytes known as natural killer (NK) cells. It typically starts in the nose or sinuses, but sometimes it starts in the skin.
It has been linked to infection with the Epstein-Barr virus (EBV) and is more common in Asia and Central and South America. It tends to grow quickly.
This is a group of rare skin lymphomas that don’t fit into any of the above categories. There are several types:
These lymphomas are sometimes referred to as cutaneous B-cell lymphomas (CBCLs).
This is the most common B-cell lymphoma of the skin. It tends to grow slowly. The early lesions are groups of red pimples, nodules (lumps), or plaques (raised areas) that form on the scalp, forehead, or upper body. Less often, they are found on the legs. Sometimes the pimples grow into nodules.
This type of lymphoma is typically found in middle-aged adults. It tends to respond well to radiation therapy, and most people have an excellent outlook.
This disease is also known as primary cutaneous marginal zone B-cell lymphoproliferative disorder. It tends to grow very slowly and is usually curable. In Europe (but not in the United States), it is sometimes linked to an infection with Borrelia, the germ that causes Lyme disease.
This disease can happen at any age, although it tends to happen more often in older adults. It appears as skin lesions that are red to purplish large pimples, plaques (raised or lowered, flat lesions), or nodules (lumps) on the arms or upper body. There may be only a single lesion, but there can sometimes be a few.
This is a fast-growing lymphoma that begins as large nodules (lumps), mainly on the lower legs. It happens most often in older people and is more common in women than men. It sometimes spreads to lymph nodes and internal organs, causing serious problems.
This type of lymphoma often requires more intensive treatment. The outlook is better if there is only one lesion at the time of diagnosis.
Developed by the American Cancer Society medical and editorial content team with medical review and contribution by the American Society of Clinical Oncology (ASCO).
Alaggio R, Amador C, Anagnostopoulos I, et al. The 5th edition of the World Health Organization Classification of Haematolymphoid Tumours: Lymphoid Neoplasms. Leukemia. 2022;36(7):1720-1748.
Campo E, Jaffe ES, Cook JR, et al. The International Consensus Classification of Mature Lymphoid Neoplasms: A report from the Clinical Advisory Committee. Blood. 2022;140(11):1229-1253.
Hathuc VM, Hristov AC, Smith LB. Primary cutaneous acral CD8+ T-cell lymphoma. Arch Pathol Lab Med. 2017; 141(11): 1469-1475.
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Jacobsen E, Willemze R. Primary cutaneous large B cell lymphoma, leg type. UpToDate. 2025. Accessed at https://www.uptodate.com/contents/primary-cutaneous-large-b-cell-lymphoma leg-type on April 1, 2025.
Kadin ME. Lymphomatoid papulosis. UpToDate. 2025. Accessed at https://www.uptodate.com/contents/lymphomatoid-papulosis on April 1, 2025.
Keeling BH, Gavino ACP, Admirand J, Soldano AC. Primary cutaneous CD4-positive small/medium-sized pleomorphic T-cell lymphoproliferative disorder: Report of a case and review of the literature. J Cutan Pathol. 2017;44(11):944-947.
Rook AH, Olsen EA. Clinical presentation, pathologic features, and diagnosis of Sézary syndrome. UpToDate. 2024. Accessed at https://www.uptodate.com/contents/clinical-presentation-pathologic-features-and-diagnosis-of-sezary-syndrome on March 7, 2025.
Willemze R. Classification of primary cutaneous lymphomas. UpToDate. 2024. Accessed at https://www.uptodate.com/contents/classification-of-primary-cutaneous-lymphomas on March 7, 2025.
Willemze R. Primary cutaneous T cell lymphomas, rare subtypes. UpToDate. 2025. Accessed at https://www.uptodate.com/contents/primary-cutaneous-t-cell-lymphomas-rare-subtypes on April 1, 2025.
Last Revised: May 19, 2025
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