Lymphomatoid Papulosis - Causes, Symptoms and Treatment

Definition

Lymphomatoid papulosis is a rare skin disorder that is characterized by crops of self healing skin lesions that look cancerous under the microscope but are actually benign. It is a chronic papulonecrotic or papulonodular skin disease with histologic features suggestive of a malignant lymphoma. There is a wide age range but the disease is most common in the 5th decade. The typical presentation is an asymptomatic red papule or nodule which may ulcerate or crust to leave a scar. The primary lesions often occur in crops and may take 3 weeks to months to resolve. These crops may number in the hundreds. The atypical cells resemble Reed-Sternberg cells of Hodgkin's disease or the malignant cells of cutaneous T-cell lymphoma. In some cases, lymphomatoid papulosis progresses to lymphomatous conditions including mycosis fungoides, Hodgkin's disease, cutaneous T-cell lymphoma, or Ki-1 lymphoma. It may occur anywhere on the body but most often affect the chest, stomach, back, arms and legs.

Causes

1. The cause of this disease is not known but it has been associated with T-cell lymphocytes.
2. T-cell lymphocytes are caused as long-term exposure to industrial or environmental metals, organic solvents, chemical carcinogens, pesticides and herbicides.
3. In some people lymphomatoid papulosis turns into lymphoma which is a form of cancer. This happens in about one out of ten people.
4. Lymphomatoid papulosis may begin as a chronic, reactive, polyclonal lymphoproliferative phenomenon that sporadically overwhelms host immune defenses and evolves into a clonal, antigen-independent, true lymphoid malignancy.

Symptoms

1. Unless accompanied by systemic lymphoma, physical findings are limited to the skin and, very rarely, the oral cavity.
2. Most patients present with multiple skin papules that may occur anywhere on the body but most often affect the chest, stomach, back, arms and legs.
3. The bumps may appear in crops and may be mildly itchy.
4. The bumps spontaneously heal with or without scarring within 2-8 weeks of appearing.
5. These bumps may develop into blood or pus-filled blisters that break and form a crusty sore before healing completely.
6. Other symptoms may be fever, anemia or fatigue depending on the condition and severeness of disease.

Treatment

1. Localized mildly pruritic skin lesions may be treated with mid- to high-potency topical steroids to hasten resolution.
2. Methotrexate is a safe and effective treatment for this disease but the disease recurs within 1-2 weeks after discontinuing the medication.
3. Phototherapy: In this treatment electromagnetic radiation can be used to treat the skin disorders. It is an effective treatment in lymphomatoid papulosis. But it is used if the topical steroids are not useful.
4. Ultraviolet treatment may also used to treat this disease. This is again an effective technique in curing this disease.
5. Patients require long-term follow-up, preferably twice yearly, to monitor the disease and evaluate for the development of associated lymphoma. Patients on systemic therapy phototherapy require closer monitoring for adverse effects.