You have been diagnosed with a condition called Mycosis Fungoides which is a type of skin lymphoma. In the north east of England we have a specialist clinic for patients with this rare type of skin cancer which is held on a monthly basis in Newcastle-upon-Tyne. This information sheet provides a summary of the condition and provides links to other information and local service contacts.
What is skin lymphoma?
Lymphoma is a cancer that begins in cells called ‘lymphocytes’ which are a vital part of our immune system. These cells are usually found in blood and lymph nodes (glands) but also in other parts of the body such as the skin, gut and spleen.
A lymphoma develops if lymphocytes grow out of control or don’t die off after their normal life span. These lymphocytes can build up and form a cancerous collection of cells. Cutaneous lymphoma develops when the lymphoma starts in the lymphocytes in the skin (‘cutaneous’ means anything related to the skin). This does not usually affect the internal organs.
There are many different types of lymphoma but they can be divided into two main groups, Hodgkin and Non-Hodgkin lymphoma. These differ in how they develop, how they behave and in how they are treated. Skin lymphomas are a type of Non-Hodgkin lymphoma.
Lymphomas starting in the skin account for around 1 in 40 of all Non-Hodgkin lymphomas and these primary ‘skin lymphomas’ are therefore quite rare. If a lymphoma starts somewhere else in the body such as the lymph nodes, then spreads to the skin, it is not considered to be a skin lymphoma.
Skin lymphomas can develop from either of the two types of lymphocytes in the body; these are called T lymphocytes (T-cells) or B Lymphocytes (B-cells). Most skin lymphomas develop from T-cells and are known as primary cutaneous T-cell lymphoma or CTCL for short. Mycosis Fungoides is the most common type of CTCL.
Why do I have skin lymphoma?
The cause of skin lymphoma is unknown. It can occur in anyone, but is more common in men, people whose immune system isn’t working properly (e.g. taking medicines which suppress it) and older people. It cannot be inherited and cannot be caught or passed to other people. Nothing you have done will have caused the lymphoma.
How is skin lymphoma diagnosed?
Many skin lymphomas look very similar to common skin conditions such as eczema or psoriasis, and making the diagnosis is often not straightforward. Many develop very slowly, some taking up to 40 years. It can therefore be months or even years before you are referred to a dermatologist and the condition is diagnosed. Such a delay is unlikely to affect the outcome of your disease.
Skin lymphoma is diagnosed by a skin biopsy however the diagnosis is not always easy and sometimes patients need more than one biopsy over a period of time before the diagnosis can be established.
What tests do I need to have?
Skin lymphomas are normally assessed by examination of the skin, feeling for lymph glands, routine blood tests and the results of the skin biopsy. It is rare for skin lymphomas to involve internal organs and therefore scans may not be required in early stage disease. Patients with more advanced stage disease (such as raised skin lumps or tumours, swollen lymph glands or abnormal blood tests) will require scans to exclude involvement of other organs.
What do the ‘stages’ mean?
The stage of the lymphoma is an indication of how much the lymphoma is affecting the body.
There are four stages of skin lymphoma; 1 to 4, although these are further broken down into A and B for each stage. Stages 1A, 1B, and 2A are considered early stage disease and 2B, 3 and 4 more advanced.
Stage 1
Red patches or raised areas (plaques) on the skin. This is divided into stage 1A, where less than 10% of the body is affected, and stage 1B, where more than 10% is affected.
Stage 2A
Similar to stage 1A, but with some enlarged lymph nodes
Stage 2B
May have any of the above, but will also have one or more skin tumours
Stage 3
At least 80% of the skin is red (erythroderma)
Stage 4A
Any of the above with lymphoma cells in the lymph nodes and/ or a high number of lymphoma cells in the bloods
Stage 4B
Any of the above, but the lymphoma has also spread to involve other organs such as the liver.
What is the prognosis for skin lymphoma?
The outlook or ‘prognosis’ depends on your age and general health and the stage of your disease. The majority of skin lymphomas present at an early stage and are associated with a normal life expectancy. Your individual circumstances can be discussed with you in clinic.
How is Mycosis Fungoides Treated?
As Mycosis Fungoides is often slow growing, the majority of patients will have no treatment (watch and wait) some of the time, with symptomatic ‘skin directed’ treatment when their condition is more active. Patients with more advanced disease may need treatment to keep their skin lymphoma under control.
‘Skin directed’ treatments include
- Emollients (moisturisers)
- Topical steroids
- Phototherapy (light treatment with either ultraviolet B (UVB) or
psoralen and ultraviolet A (PUVA))
- Radiotherapy
Treatment for more ‘advanced disease’
- Immunotherapies, such as interferon alpha and bexarotene
- Chemotherapy
- Extracorporeal photophoresis
- Steroid tablets
- Haematopoietic stem cell transplant
Further information on specific treatments or your possible involvement in a clinical trial can be discussed with you.
Follow-up and contact details
You will be offered follow up in the long term in the CTCL clinic in Newcastle upon Tyne. If you are concerned or your condition changes between appointments then please contact us for an earlier appointment by contacting the consultant dermatology secretaries on 0191 2824340 or specialist nurse team on 0191 2824110.
Further Information
Lymphoma Association
Freephone helpline 0808 808 5555
