Primary cutaneous marginal zone lymphoma (PC-MZL) is a B-cell lymphoma arising in the skin. Although it is a rare disease, PC-MZL accounts for 20-40% of all primary cutaneous B-cell lymphoma in Western Countries. The aetiology and the pathogenesis of PC-MZL are poorly understood, as it generally lacks the chromosomal translocations most typically present in marginal zone lymphomas of other sites. The diagnosis of PC-MZL may be challenging, due to the rarity of the disease, and needs the competence of different professional figures, including the dermatologist and the pathologist. Furthermore, the management of the patient after the diagnosis is complex and involves the dermatologist, the haematologist, the surgeon, the radiotherapist and the radiologist. The aim of this review is to describe the clinical and histological findings for the diagnosis of PC-MZL, and the state of art for the management of the patient.
Keywords: Borrelia burgdorferi; cutaneous lymphoma; dermoscopy; immunohistochemistry; marginal zone lymphoma.
© 2021 Ronchi et al.