Lymphomatoid papulosis is a rare skin disorder with cases reported only in 1-2 people per one million. Papules develop on the skin usually on a person’s trunk.
Papules are a raised area of the skin that are usually pink, but can be other colors such as skin tone, brown, red, or purple. In lymphomatoid papulosis the papules slowly develop in groups on someone’s skin. Then over the next six to 12 weeks they go away.
Most patients experience no other symptoms and the cause of lymphomatoid papulosis is unknown. Researchers do know that lymphomatoid papulosis has the potential to evolve into lymphoma so the focus of treatment is to prevent this from happening.
How does it work?
Treatments for lymphomatoid papulosis work by speeding up the process of the papules going away and thus hopefully preventing the lymphomatoid papulosis from developing into anything worse. It is recommended that patients have an evaluation by a dermatologist with experience in cutaneous lymphoma.
The dermatologist can do a biopsy of the papules and make a diagnosis. It can also be beneficial to see a dermapathologist who will evaluate the cells under a microscope.
Currently there are three main types of treatment for lymphomatoid papulosis. These are topical medications, oral medications and phototherapy. Topical medications include steroids and the main aim of this type of treatment is to decrease the length of time it takes for the papules to go away.
Oral medications include a group of drugs known as antimetabolites. These inhibit the cells from growing into malignant cells. Phototherapy, when used in combination with oral medication, has also proven successful. Traditionally treatment for lymphomatoid papulosis has been weekly low doses of methotrexate.
Methotrexate has been successful in treating the symptoms and the disease; however, papules return very soon after discontinuing this treatment.
Since lymphomatoid papulosis is so rare it has only been studied since the late 1960’s. Other medications that have been used and show promise for lymphomatoid papulosis treatment include topical agents such as nitrogen mustard, imiquimod cream, intralesional interferon, and carmustine.
Since lymphomatoid papulosis presents in such noticeable way on the skin most patients are diagnosed early and treated accordingly. It is recommended that patients be monitored at least twice a year to ensure that it is not developing into lymphoma. Patients receiving phototherapy or weekly treatments are obviously monitored very closely. There are no reports of casualties from this disease. Prognosis is excellent.