Primary Cutaneous Marginal Zone B-cell Lymphoma (PCMZL)
Definition
Primary Cutaneous Marginal Zone B-cell Lymphoma (PCMZL) is the second most common form of primary cutaneous B-cell lymphomas. It is associated with non-Hodgkin lymphomas, specifically with the extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT type). Primary cutaneous B-cell lymphomas account for approximately 20–25% of all cutaneous lymphomas and generally present with an indolent clinical course, slow progression, and absence of severe symptoms. • Specialized diagnosis • Accurate imaging and staging • Personalized therapeutic approach • Progressive management • Regular follow-up to prevent complications
Symptoms
This type of lymphoma can manifest on any area of the skin, but it typically appears on the arms, legs, or trunk. The clinical presentation includes papules, nodules, or—more rarely—tumors. In some cases, patients may show signs of infection with Borrelia burgdorferi, the bacterium associated with Lyme disease. Characteristic lesions appear as erythematous to violaceous papules, plaques, or nodules, mainly on the upper limbs or trunk, and may recur over time. Borrelia burgdorferi is considered a potential contributing factor. Extracutaneous spread or spontaneous regression is rare.
Diagnosis & Treatment
The average age of onset is approximately 63 years. Clinical presentation may be atypical, such as cystic masses or pseudodermatitis, making diagnosis more challenging. For solitary lesions, surgical excision or radiotherapy is typically recommended. In more widespread or multifocal presentations, Rituximab is often the treatment of choice. Due to the benign nature and favorable prognosis of PCMZL, a “watch-and-wait” strategy is often employed as first-line management. However, proper staging using imaging methods such as computed tomography (CT) is essential to rule out systemic dissemination and secondary skin infiltration. Overall, diagnosis and treatment require expert dermatologic evaluation and careful case-by-case management.
