Mycosis fungoides: symptoms, stages, treatment and outlook

BAD says the most common symptom is long-term, generalised itching (pruritus), and rarely there may be pain in the affected areas. The rash usually develops slowly. At first, irregularly shaped dry patches appear (the patch stage), often on skin that is not usually exposed to the sun, which may look like eczema or psoriasis.

Lymphoma Action adds that patches are dry, sometimes scaly and may be itchy, most common on the buttocks or torso but possible anywhere, and that they can be lighter or darker than surrounding skin on Asian or black skin. In most people the lymphoma never develops beyond the patch and plaque stage.

Mycosis fungoides is a lymphoma, a cancer of lymphocytes (white blood cells), that starts in the skin. BAD explains that T-cells normally help the body's germ-fighting immune system, and in cutaneous T-cell lymphoma they develop abnormalities that make them attack the skin.

Newcastle Hospitals says skin lymphomas account for around 1 in 40 of all non-Hodgkin lymphomas, so they are rare, and that most develop from T-cells. Lymphoma Action says skin lymphoma is not a type of skin cancer in the sense of developing from skin cells, and that it is not linked to sun exposure.

Skin biopsies are usually needed. BAD explains that a small sample of skin is taken under local anaesthetic and examined under a microscope. Because the rash and biopsy may show no specific features and can resemble other skin conditions, biopsies may be repeated several times, and blood test results are usually normal.

Newcastle Hospitals adds that skin lymphomas are assessed by examining the skin, feeling for lymph glands, routine blood tests and the skin biopsy result. Scans may not be needed in early stage disease, but are used with more advanced disease, such as raised lumps, swollen lymph glands or abnormal blood tests. It says delay in diagnosis is unlikely to affect the outcome.

Newcastle Hospitals describes four stages, 1 to 4, each broken down into A and B. Stages 1A, 1B and 2A are considered early stage disease, and 2B, 3 and 4 more advanced.

BAD says the aim of treatment is to control symptoms and improve quality of life, and that no treatments have been shown to improve survival, although with early diagnosis people often live for many years without symptoms. Periods with no treatment are common, and Newcastle Hospitals says most patients have "watch and wait" some of the time.

Good skin care with regular moisturisers (emollients) is part of the plan. Most people only ever need skin-directed therapy: steroid creams or ointments, chlormethine gel, and phototherapy. If that does not control the condition, the case is discussed by a specialist skin lymphoma multidisciplinary team and other treatments may be offered.

BAD says there is no known cure, but treatment often clears the rash for some time, and even after effective treatment the condition usually comes back. It is a life-long condition that usually progresses slowly over many years, and it is difficult to predict how it will affect each person.

The outlook depends on your age, general health and the stage of the disease. Newcastle Hospitals says the majority of skin lymphomas present at an early stage and are associated with a normal life expectancy, while BAD says most affected people have a normal life span but people with a more advanced stage may have worse outcomes.

The cause is unknown. BAD says you cannot catch or pass on mycosis fungoides, it is not an infection and it is not hereditary, with very rare cases of several family members affected. Newcastle Hospitals adds that it is more common in men, in older people and in people whose immune systems are not working properly, such as those taking medicines that suppress it, and that nothing you have done will have caused it.

BAD says there will usually be no restrictions on day-to-day activities and no special diet is needed. Moisturising regularly is worth trying with different creams or ointments until you find one that suits you, and many moisturisers are available on prescription.

Newcastle Hospitals says you will be offered long-term follow-up in a specialist cutaneous lymphoma clinic and can ask for an earlier appointment if your condition changes. Support groups include Lymphoma Action and the Cutaneous Lymphoma Foundation, which BAD lists.

On the NHS the route is a GP or dermatologist, then a specialist skin lymphoma clinic and the regional skin lymphoma multidisciplinary team (BAD says there are several across the UK). Treatment decisions for this rare condition are made by that specialist team.

Private dermatology can provide an earlier first opinion and biopsy, but ongoing treatment, including phototherapy and radiotherapy, is usually coordinated through the NHS specialist service. Ask who will be your main contact.