Lymphomatoid papulosis

You have been diagnosed with a condition called lymphomatoid papulosis. In the North East of England we have a specialist clinic for patients with this rare type of skin disease as well as those with skin lymphoma. This 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 lymphomatoid papulosis?

Lymphomatoid papulosis is a skin disorder that often comes and goes on its own without treatment. These lesions may appear in crops (groups) and can ulcerate in the centre, and sometimes heal leaving scars. These lesions look cancerous under the microscope but are actually benign (non-cancerous).

They contain cells that look like those found in some lymphomas (cancers of the lymphatic system), but is not fatal and does not spread to internal organs.

Although lymphomatoid papulosis is a benign condition, approximately 15% of patients have an associated lymphoma either before, at the same time as, or after the diagnosis. This may be T-cell lymphoma affecting just the skin (which can present as scaly patches sometimes mistaken for difficult to treat eczema) or a more serious internal form of the disease.

What causes lymphomatoid papulosis?

This is not known, but it is associated with an expansion of atypical T-cells (unusual-looking white blood cells). It can affect both younger and older people. The average age of diagnosis is 45 years. It is a rare disorder, occurring in about 1.2-1.9 people per million. It is more common in men and affects people of all races and age, although it is less often seen in people with black skin.

What are the signs and symptoms?

Patients develop anything from one to hundreds of raised lesions, which commonly occur in crops and may appear at any part of the body. These may be mildly itchy. Lesions may develop into blisters that ulcerate (top layers of skin come away), forming a central black scab before healing completely.

They heal without being treated in approximately 2-8 weeks, although crops may recur regularly over many years. The condition can settle completely over months or years, but may come back in the future.

How is the diagnosis made?

Lymphomatoid papulosis is diagnosed using a combination of clinical assessment and skin biopsy; although sometimes several biopsies are required before a diagnosis can be confirmed.

The diagnosis is sometimes confused with other skin conditions that can look similar, such as acute pityriasis lichenoides.

What treatment is available?

Lymphomatoid papulosis often lasts for years, and there is no ‘cure’ that can be given. Several treatments can help reduce healing time or prevent new lesions from appearing. These include:

Topical steroid creams/ointments.
Phototherapy with UVB (ultraviolet B treatment) or PUVA (psoralen tablets followed by ultraviolet A treatment).
Low dose methotrexate which is taken once weekly, and can be highly effective in some patients.

Long term follow-up is recommended to improve detection of skin or internal lymphomas which are more serious and occur in 15% of affected patients.

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 282 4340 or specialist nurse team on 0191 282 4110.