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Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare form of lymphoma that causes skin lesions (sores). Because SPTCL is so uncommon, there aren’t official guidelines on how to diagnose or treat it. Keep reading to learn about the symptoms, how doctors diagnose it, and the treatments available.
SPTCL is a type of peripheral T-cell lymphoma (PTCL). To better understand this type of cancer, we’ll break down each word, starting from the end with lymphoma.
Lymphoma is a type of blood cancer where infection-fighting white blood cells called lymphocytes develop abnormally, crowding out healthy cells. Hodgkin lymphoma (HL) and non-Hodgkin lymphoma (NHL) are the two main types of lymphoma. Both HL and NHL are cancers of the lymphatic system — part of the body’s immune system that protects the body by removing bacteria, waste matter, and excess fluids from old or damaged cells. SPTCL is a form of NHL.
T cells are a type of lymphocyte. There are two kinds of lymphocytes — T lymphocytes (or T cells) and B lymphocytes (also called B cells). Lymphoma can develop in either T cells or B cells.
SPTCL develops in T cells. When a T-cell lymphoma develops in the skin, it’s known as a cutaneous T-cell lymphoma (CTCL). The T cells in SPTCL have a unique genetic mutation (change, or variation) compared to other types of CTCLs. Lymphomas of the skin may also begin in B cells — these are called cutaneous B-cell lymphomas.
As its name suggests, SPTCL often looks like another condition: panniculitis.
Panniculitis is a condition that causes inflammation of the fat layer beneath the skin (called subcutaneous fat). This inflammation can lead to symptoms such as skin discoloration, tenderness or pain, and the formation of nodules (firm bumps) under the skin. In some cases, panniculitis may also cause whole-body symptoms like fever, joint pain, and fatigue. In SPTCL, abnormal T cells can cause inflammation that looks like panniculitis.
Subcutaneous refers to the layer of fatty tissue under the skin. That’s where subcutaneous lymphomas form. SPTCL doesn’t usually involve the top layers of the skin.
SPTCL is an extremely rare form of cancer, making up less than 1 percent of all non-Hodgkin lymphoma diagnoses. According to StatPearls, the average age when people develop SPTCL is around 36, and it’s slightly more common among females than males.
About 20 percent of the time, SPTCL is linked to an autoimmune condition, especially systemic lupus erythematosus (SLE, the most common form of lupus). An autoimmune disease happens when the immune system, which normally fights off germs, mistakenly attacks healthy cells. People with lupus are more likely to develop SPTCL, and people with SPTCL are more likely to have lupus. Researchers don’t yet know how these two conditions are connected or if one leads to the other.
Most people with SPTCL have symptoms that only affect the skin. However, other symptoms can occur in about half of people with this condition.
The main symptom of SPTCL is a series of nodules under the skin. Because they form in the subcutaneous tissue, these nodules may feel like they are deep under the skin. Few people report that these nodules are painful.
Subcutaneous nodules can form on the arms, legs, or trunk of the body. They are small — between half a centimeter and 2 centimeters in diameter — and may come and go. In rare cases, the lumps under the skin can ulcerate (break open).
SPTCL nodules may seem to appear and disappear at random, especially in the early stages of the condition. As SPTCL progresses, the nodules may linger for a longer time or appear over multiple areas of the body simultaneously. Most of the time, SPTCL doesn’t involve the lymph nodes or bone marrow.
Approximately 50 percent of people diagnosed with SPTCL also experience systemic signs and symptoms — also known as B symptoms — which include:
About 15 percent to 20 percent of people with SPTCL develop hemophagocytic syndrome (HPS). HPS is a life-threatening complication that causes overwhelming inflammation and multiple organ failure. This reaction is tied to the Epstein-Barr virus (the virus responsible for mononucleosis), which can also play a role in some autoimmune diseases. Research into connections between this virus and SPTCL is ongoing.
Because SPTCL can look like other conditions (such as eczema, dermatitis, and prurigo nodularis), it can take months or years to diagnose it accurately. A differential diagnosis — one that rules out other causes — can help determine whether your symptoms are caused by SPTCL or another commonly confused condition, like lupus.
Diagnosis of SPTCL may include procedures commonly used to diagnose non-Hodgkin lymphoma. Your doctor will use the combined results of these tests and exams to make a diagnosis.
A doctor will examine your skin to look for signs of subcutaneous nodules. They may also look for other symptoms of lymphoma and ask whether you’ve had systemic symptoms, such as unexplained fevers, chills, and unintended weight loss.
A dermatologist or dermatologist-oncologist will perform a skin exam. A dermatologist-oncologist is a doctor who specializes in both skin conditions and skin cancers. During the exam, they will check to see if the lumps under the skin are caused by something other than lymphoma.
A skin biopsy is a test that removes a sample of the skin to test in a laboratory. A punch biopsy is most often used to help diagnose SPTCL. In a punch biopsy, the dermatologist uses a special cutting tool to remove a section of affected skin that includes the skin and deeper layers of the subcutaneous fat. Several biopsies may be needed to accurately diagnose SPTCL.
If the lump is very large, your doctor may suggest an excisional biopsy where tissue is removed with a scalpel. Shave biopsies that only take a sample of the top layers of skin likely won’t be enough to diagnose SPTCL.
A pathologist will examine the punch biopsy under a microscope to check for signs of SPTCL. One key feature is abnormal T cells surrounding fat cells. The pathologist can also test for genetic mutations in any cancer cells.
Doctors may take a sample of your bone marrow and examine it under a microscope to check for lymphoma cells. This test can help show whether the lymphoma has spread. It can also help doctors decide on the best treatment plan.
Your doctor may examine your nodules and fatty tissue cells using a computerized tomography (CT) scan, magnetic resonance imaging (MRI), or ultrasound technology. They may also perform a positron emission tomography (PET) scan to look for nodules in other areas of your body.
Blood tests can be used to directly test for lymphoma cells and monitor your overall health and symptoms. Blood tests to monitor your health may include:
Because SPTCL often occurs alongside lupus, your doctor may look for signs of an autoimmune disorder. They might order blood tests to check for markers of inflammation or other signs that your immune system is overactive. Finding an autoimmune condition can help your doctor choose the right treatment.
Staging describes how advanced a case of cancer is. When SPTCL is diagnosed, your doctor will do their best to stage the cancer by checking whether the lymph nodes are affected. Most cases of SPTCL are diagnosed at an early stage.
Because SPTCL is so rare, it can be hard for doctors to figure out how far it has spread. Researchers also don’t fully understand how the disease usually develops over time. Your medical team will do their best to accurately stage the cancer and determine the right course of treatment.
Treatment options for SPTCL can vary based on your symptoms and where the nodules are. Because the condition is so rare, treatments for SPTCL have not yet been standardized. Treatment recommendations are based on case reports and case series that describe the diagnosis and treatment of people with SPTCL.
Below, we’ll review common treatment options.
Corticosteroids (steroids) and immunosuppressants play an important part in the treatment of SPTCL. These drugs help to dial down the immune response that seems to play a part in the progression of the disease.
Prednisone is an example of a steroid medication used for SPTCL. Steroids can be used with or without other drugs, such as immunosuppressants and chemotherapy. In some cases, prednisone is combined with bexarotene. Bexarotene is a type of retinoid, a drug related to vitamin A, that helps slow the growth of T cells. Immunosuppressants used to treat SPTCL include cyclosporine A and methotrexate.
Chemotherapy isn’t a first-line (preferred) treatment option for SPTCL. However, it may be used in people with progressive disease. A common chemotherapy regimen used for SPTCL is CHOP, which includes the following drugs:
In some cases, etoposide is added to the CHOP regimen.
Radiation therapy is a treatment that uses radiation to kill cancer cells. This treatment may be an option for people whose disease is limited to one area and hasn’t spread.
Another approach for treating SPTCL is surgery to remove nodules that cause discomfort or that are deep in subcutaneous tissue. However, this is uncommon, and may only be an option for those with localized disease.
A bone marrow transplant may be considered when other treatments don’t work or when the cancer has spread beyond the skin. This procedure uses healthy immature cells from a donor to replace lymphoma cells. Research is still underway to better understand how well this works for SPTCL.
If standard treatments aren’t effective, joining a clinical trial may give you access to new therapies, like immunotherapy or targeted drugs, that are still being studied. Talk to your healthcare provider to see if there are any trials near you.
Most cases of cutaneous lymphomas are caught in the early stages, so people have a chance to get the treatment they need before the cancer progresses very far. The prognosis (outlook) for people diagnosed with SPTCL is generally positive. The overall five-year survival rate is between 85 percent and 91 percent for those with primary cutaneous forms of the condition.
People who also develop the serious complication HPS may have a worse prognosis. Only about 46 percent of people with SPTCL complicated by HPS are still alive after five years. Recent research has also found that people with symptoms on their arms may have a worse prognosis.
If you’ve been diagnosed with SPTCL or are noticing symptoms, talk to your doctor about your concerns. They can help explain your test results, discuss treatment options, and answer any questions you have. Staying informed and working closely with your healthcare team can help you feel more confident about managing your health.
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Thanks for info...CLL has been kicking my but, swelling, pain, occasional fevers, chills, sweats at times in bed. Liver enzyems went out of control, liver and spleen swelling, hospital stay with… read more