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Author: Vanessa Ngan, Staff Writer, 2003.
Lymphomatoid papulosis is a rare skin disorder that is characterised by crops of self healing skin lesions that look cancerous under the microscope but are actually benign (non-malignant). Lesions contain unusual cells that are similar to those found in some lymphomas (cancers of the lymphatic system).
Although most cases of lymphomatoid papulosis are benign, 10-20% of patients may have an associated lymphoma before, at the same time as, or after the diagnosis of lymphomatoid papulosis.
The cause of lymphomatoid papulosis is unknown but it is associated with a proliferation of atypical T-cell lymphocytes (funny-looking white cells). The estimated incidence is 1.2-1.9 cases per million population so it is a rare disease. It may affect people of all races, sex and age but is less often seen in black skinned people.
Most patients present with multiple skin papules (raised bumps) that may occur anywhere on the body but most often affect the chest, stomach, back, arms and legs. The papules appear in crops and may be mildly itchy. They are quite variable in appearance. They may develop into blood or pus-filled blisters that break and form a crusty sore before healing completely. Lesions spontaneously heal with or without scarring within 2-8 weeks of appearing.
The number of lesions present can vary from one to hundreds. Crops of lesions come and go over many years.
Four histopathologic variants have been described: Type A, Type B, Type C and Type D. Some patients have more than one type of lesion. Specific tests evaluate the types of T-cell lymphocytes in the infiltrate: CD30+ cells are characteristic of lymphomatoid papulosis.
The following treatments are used to hasten healing of lymphomatoid papulosis lesions and/or suppress the disease.
There is no cure for lymphomatoid papulosis. It often lasts for years, over which time patients need to regularly see their doctor to pick up any changes that may signal early lymphoma.
Other forms of lymphoma have been reported to follow lymphomatoid papulosis in 4–25% patients, so long term follow-up is recommended. These incldue:
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