Primary cutaneous follicle center lymphoma

Primary cutaneous follicle center lymphoma (PCFCL) is a type of indolent (slow-growing) non-Hodgkin B-cell lymphoma that originates in the skin without evidence of extracutaneous disease at the time of diagnosis. It is one of the most common primary cutaneous B-cell lymphomas and belongs to the group of cutaneous follicle center lymphomas classified under the WHO-EORTC classification. The disease is also sometimes referred to as primary cutaneous follicular lymphoma or cutaneous follicle center cell lymphoma. PCFCL typically presents as solitary or grouped, firm, reddish to violaceous papules, plaques, or nodules, most commonly located on the scalp, forehead, or trunk (particularly the back). The lesions tend to grow slowly over months to years. Unlike systemic follicular lymphomas, PCFCL generally has an excellent prognosis with a 5-year disease-specific survival rate exceeding 95%. The disease predominantly affects the skin, though in rare cases it may disseminate to extracutaneous sites, particularly lymph nodes. Treatment depends on the extent and distribution of skin lesions. For solitary or localized disease, local radiation therapy is considered the treatment of choice and is highly effective. Surgical excision may also be used for small, isolated lesions. For patients with multifocal skin involvement, options include rituximab (an anti-CD20 monoclonal antibody) administered either systemically or intralesionally, combination immunochemotherapy, or a watch-and-wait approach in selected cases. Importantly, PCFCL does not typically express BCL2 protein, which helps distinguish it from secondary cutaneous involvement by systemic follicular lymphoma. Regular follow-up is recommended to monitor for recurrence, which can occur locally but rarely affects overall survival.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

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