Erythema multiforme-like lesions in primary cutaneous aggressive cytotoxic epidermotropic CD8+ T-cell lymphoma: a diagnostic and therapeutic challenge

Primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma (pCAECD8+ CTCL), is an extremely rare, rapidly progressing cutaneous lymphoma, with frequent systemic involvement, first recognized as a distinct clinicopathologic entity by Berti et al in 1999. (1) As the name suggests, this entity has an aggressive behavior and exhibits marked epidermotropism on histopathologic analysis. Conventional treatment modalities for classic CTCL are often ineffective and the prognosis is poor with a median survival of 12 months. (2,3).