Clinical Features

Sezary syndrome is an uncommon CTCL which accounts for less than 5% of CTCL (4,5). Sezary syndrome is characterized by distinct clinical features with erythroderma accompanied by intense and generalized pruritus, diffuse alopecia, palmoplantar hyperkeratosis and onychodystrophy as well as lymphadenopathy (Figs. 1-3). In the peripheral blood, lymphoid cells with hyperconvoluted nuclei (so-called Sezary or Lutzner cells) of varying size (8 to > 12 micrometer in diameter) and number are found (6). Interestingly, clinicopathologic manifestations of SS

Lutzner Cells

Figure 1 Erythroderma with striking lymphoadenopathy and edema of the lower extremities.

Thick Skin Figure
Figure 2 Massive edema (anasarca) creating thick skin folds on the back.

other than the classic one seem to be very rare or not yet identified. Granulomatous lesions harboring clonal T-cells in a patient with long-standing SS were recently described (7).

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