Lymphomatoid Papulosis

Definition

This is a chronic disease. It happen on the skin of the affected person. It is characterized by spontaneously regressing papules composed of an atypical CD30-positive T-cell population. Lymphomatoid papulosis is a stubborn persistent rash that usually occurs on the chest, stomach, back, arms and legs.

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.

Around ten to twenty percent of the patients might have an associated lymphoma before. The bumps that are formed heal within two to three weeks but they reappear. The rashes formed continue for the rest of the life as up till now no cure has been discovered for this skin disorder.

Causes

The cause of lymphomatoid papulosis is not known.

Genetic predisposition is the main cause, but other causative agents such as long-term exposure to industrial or environmental metals, organic solvents, chemical carcinogens, pesticides and herbicides are also under consideration from the medical researcher. It is really a very rare disease because it estimated incidence is 1.2-1.9 cases per million population. It may affect people of all races, sex and age but is less often seen in black skinned people.

Symptoms

• There may be appetite loss
• Fever and Anemia problem may also present.
• Infections associated with low white count
• Drenching night sweats
• Pain :depending on the anatomic location that is secondary to pressure to bone or organs, or obstruction of bodily functions
• Skin - Itchy skin (purititis), red patches
• Swollen and painless lymph nodes
• Weight loss also occur in this disease.

Treatment

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.

In the initial stage, a cyclic treatment method is done. In the next treatment method, a total skin electron beam (TSEB) radiation therapy is done. TSEB radiation therapy uses special high-energy rays of tiny electron particles to kill the cancer cells. The effects of this therapy will kill the existing tumors and allow the healing of the ulcerations. This therapy usually lasts only a few months. During this remission period, a second treatment method of topical chemotherapy (skin drugs) is introduced.

Skin biopsy needs to be conducted in order to diagnose this disease and in some cases it needs to be repeated on several occasions. It has been found out that this disorder generally appears between the ages of 26 and 34 although it can strike people of all ages.