It is becoming increasingly evident that primary cutaneous lymphomas represent distinct clinical and histopathological subtypes of extranodal lymphomas [1-7]. They can be defined as neoplasms of the immune system, characterized by a proliferation of either T or B lymphocytes which show a particular tropism for the skin. Extracutaneous spread with lymph node involvement can be observed during the course of the disease.

Primary cutaneous lymphomas should be separated from secondary skin manifestations of extracutaneous (usually nodal) lymphomas, which represent metastatic disease and are characterized by a different prognosis and treatment. Because the histopathology of primary and secondary cutaneous lymphomas may be similar or identical [8], complete staging investigations are needed to establish this distinction.

The standard definition of primary cutaneous lymphomas is disease confined solely to the skin for at least 6 months after complete staging procedures have been performed [4]. Recently, two of the authors (LC, HK) proposed changing the definition to 'no extracutaneous manifestations of the disease at presentation' [7]. The main reason for adopting this definition is the need to classify the disease accurately, and treat patients accordingly, at presentation, without waiting for 6 months.

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