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Lymphomatoid papulosis is a variety of cutaneous lymphoma, meaning that it is an abnormal proliferation of a type of white blood cells called lymphocytes. It is, however, said to be a “low grade” lymphoma, which means that it is not very aggressive.
WHAT ARE THE SYMPTOMS OF THE DISEASE?
Patients affected by the disease present with red papules (small “bumps”) that change their appearance after a few days becoming covered with scales (the skin sheds in small flakes), which is followed by reddish-black crusts, before finally spontaneously disappearing and leaving behind a temporary brown mark or in some cases, a small scar in the form of a pit similar to those seen after chicken pox. There is a great deal of variation in the numbers of papules, ranging from one or two to several dozen and they appear in flare-ups interspersed with periods of remission. During flare-ups, there may be itching. There are no other symptoms, such as fever, pain or tiredness. Any part of the body can be affected and, in very rare cases, the mucosae (mouth, genital regions) can be affected.
Flare-ups occur repeatedly, varying in their intensity and in how often they occur, over the course of some years although spontaneous resolution is possible.
HOW IS IT DIAGNOSED?
This is a rare skin disorder, one that very few general practitioners will encounter in their day-to-day practice. A dermatologist will be able to make a diagnosis quickly by asking you questions and looking at your skin lesions. In order to confirm the diagnosis, there will be a biopsy of one of the lesions under local anaesthetic, and this will be sent to a pathology laboratory to be examined under the microscope.
ONCE A DIAGNOSIS IS MADE, WHAT THEN?
No further examinations (blood test, ultrasound, CT scan etc.) are needed. In fact, although this is a lymphoma, lymphomatoid papulosis only affects the skin. However, studies show that in some cases lymphomatoid papulosis can, after a course of several years, be associated with other types of lymphoma, mainly varieties that affect the skin and that are themselves not very aggressive. This means that you will need to see your dermatologist once a year in order to screen for the earliest signs, such as permanent red plaques or “bumps” that are larger than the papulosis bumps and, most importantly, which do not tend to disappear after a few weeks. Your dermatologist will be able to advise you about these early signs so that after two or three years, especially if your lymphomatoid papulosis is not particularly active, you will be able to space out your appointments while being able to watch out for signs yourself.
The eventuality of a secondary lymphoma is rare and all the studies have shown that lymphomatoid papulosis never causes death and it does not shorten life expectancy.
HOW IS LYMPHOMATOID PAPULOSIS TREATED?
Before starting a treatment, your dermatologist will have contacted a specialist hospital department where your medical records will be examined by doctors well-versed in both dermatology and haematology (dermatological oncology multidisciplinary meetings) so that you can be offered a treatment.
Very often, when there are not too many lesions and they do not tend to reappear on visible areas of skin, your doctor will suggest that you do not have any treatment. Not treating lymphomatoid papulosis does not increase the risk of your condition progressing to become more serious.
When treatment is needed (large number of lesions, lesions affecting the face or at your request if the disorder is causing you problems in day-to-day life), there are three treatments available:
— Applications of creams containing a cortisone derivative (also known as topical steroids or topical corticosteroids). In contrast to cortisone in tablet form, there is no health risk in applying these creams, but they must be prescribed by a dermatologist. The cream will need to be applied twice a day to papules as they appear. This “as needed” treatment leads to reduced inflammation but it doesn’t stop other lesions from appearing.
— Phototherapy sessions. I These are sessions lasting a few minutes that take place in a cabin that delivers ultraviolet rays (a type of solar ray) to your exposed skin; you would have two or three sessions a week over a number of weeks. They are only carried out at your dermatologist’s clinic and under observation. This treatment can lead to red skin. The risk of cancer triggered by these rays is non-existent because it will be a limited number of sessions taking place over a fixed time. In general, after around fifteen sessions all or nearly all of your skin lesions should have cleared up. At that point, the dermatologist may stop the sessions or you may be offered a gradual tailing off of treatment by spacing out the sessions over a few weeks.
— Methotrexate. This treatment is suggested when phototherapy has failed or if it is not possible for you to attend the dermatologist’s clinic often enough to have phototherapy. This treatment is taken once a week in the form of tablets or intramuscular or subcutaneous injections. A vitamin tablet known as folic acid must always be taken alongside this treatment. For this treatment to be started a few laboratory tests are needed (in particular to check your kidney and liver function and your levels of red and white blood cells) that will later be repeated, as well as a vaccination against pneumococcal infection (one vaccination and a booster two months later) and a flu vaccination in winter. Patients normally do not suffer many side effects with methotrexate, although the day after it is taken some patients may suffer from mild and temporary stomach upsets. This medicine is moderately toxic for the liver, especially for patients who are overweight or drink alcohol. In very rare cases it can lead to respiratory allergic reactions. In women of childbearing age, strict contraception must be used. The treatment is continued until all your lesions clear up or only a few remain and then the dose will be gradually reduced. While this treatment usually continues for a few months, it can happen that patients need to continue to have the treatment for several years.
All treatments of any kind usually only defer the inevitable because the risk of recurrence is so great. Just as the first episode can remain untreated, there is no need to restart treatment for flare-ups.
In very rare cases, lymphomatoid papulosis can affect children. Studies show that the disorder very often cures itself spontaneously around puberty. Doctors will hesitate to prescribe sessions of phototherapy or methotrexate for a child. However, applications of steroid creams are safe. To help you choose a treatment for your child, your dermatologist will direct you to a specialist dermatology department.
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