Lymphomatoid Papulosis Follow-up
- Author: John A Zic, MD; Chief Editor: William D James, MD more...
Further Outpatient Care
Patients with lymphomatoid papulosis (LyP) require long-term follow-up, preferably twice yearly, to monitor the disease and evaluate for the development of associated lymphoma.
Patients on systemic therapy or PUVA phototherapy require closer monitoring for adverse effects.
The prognosis of lymphomatoid papulosis (LyP) is good because most patients have a chronic, indolent course. A retrospective cohort analysis found that no patients with lymphomatoid papulosis died of the disease, and the overall survival rate was 92% at 5 and 10 years.
Physicians are guardedly optimistic about the prognosis because estimates indicate that as many as 4-25% of patients have a history of associated malignant lymphoma (ALCL, HD, MF) prior to, concurrent with, or subsequent to the diagnosis of lymphomatoid papulosis.[21, 38] Unfortunately, no clinical or histologic factors analyzed to date are predictive of worse outcomes in persons with lymphomatoid papulosis. A study suggested that fascin expression is increased in lymphomatoid papulosis cases associated with a malignant lymphoma. Alterations in transforming growth factor-beta signaling are hypothesized to play a role in the progression of lymphomatoid papulosis to malignant lymphoma. Additionally, data have shown an increased risk of associated lymphomas in lymphomatoid papulosis cases with CCR3+ atypical cells or the 30M362 allelic form of the CD30 promoter.
Primary cutaneous anaplastic large cell lymphoma (pcALCL) is more likely than mycosis fungoides (MF) to manifest as an ulcerated tumor and palpable lymph nodes. MF is the most common variant of cutaneous T-cell lymphoma and is characterized by the development of red patches or plaques in sun-protected areas. MF is more likely to manifest as patches and plaques than tumors. Disease-specific survival at 5 and 10 years for pcALCL was 85% in a recent study.
Associated lymphomas more rarely include immunoblastic lymphoma, lethal midline granuloma (currently considered as natural killer cell lymphoma in many patients), and systemic lymphocytic lymphoma. In most patients, the malignancy develops many years after the diagnosis of lymphomatoid papulosis.
Instruct patients that they are not an infectious risk to others.
Instruct patients about local wound care of open crusted lesions. Lesions should be cleaned with mild soap and water twice daily, and topical petroleum ointment should be applied to prevent infection and aid healing. Crusted lesions should be covered with a loose adhesive bandages until healed.
Instruct patients to contact their physician if any of the following symptoms or signs develop that may herald the onset of infection or associated malignancy:
Fever, chills, night sweats, or weight loss
Persistent skin nodules or patches that do not regress over a 2- to 3-month period
Enlarged lymph node glands in the neck, groin, or axillae
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