Another epidermotropic form of CD8+ CTCL is often characterized by an aggressive course (5). Generalized patches, plaques, papulonodular lesions, and tumors have been described (Fig. 4); the clinical picture has been compared to disseminated pagetoid reticulosis or MF, respectively. Histologically, a band-like infiltrate of pleomorphic T-cells or immunoblasts with epidermotropism and epidermal changes with acanthosis, spongiosis, intraepidermal blistering, and necrosis are seen. The tumor cells express CD3, CD8, CD7, CD45RA, and beta F1 as well as TIA-1. A CD2- CD7+ phenotype seems to be associated with a more aggressive course (6).
An aggressive course with extracutaneous spread to CNS, visceral organs, and oral mucosa is common with a median survival of 32 months (5). Similar cases with an aggressive course have sometimes shown angiocentric and angiodestructive growth (7-9). Patients with erythroderma and peripheral blood involvement consistent with SS but also expressing CD8 have also been identified (1).
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