ABSTRACT
INTRODUCTION: A primary cutaneous CD-30 positive T -cell lymphoproliferative disorders are rare and heterogeneous group of primary skin tumors, which include primary cutaneous anaplastic large cell lymphoma (PCALCL) and lymphomatoid papulois. CASE REPORT: We report a rare an atypical case of PCALCL with an aggressive and refractory behavior that occurred in the lip vermilion, and that had been initially diagnosed as aggressive herpes. The lesion was recurrent and refractory to the CHOEP chemotherapy protocol followed by radiotherapy, but after hyper CVAD (acronym) + brentuximab with a considerable improvement. After 4 cycles of hyper CVAD plus brentuximab, the patient underwent 100% compatible sibling bone marrow transplantation, with success in the procedure. CONCLUSION: Primary cutaneous anaplastic large cell lymphoma is a rare disease of difficult diagnosis and may be confused with chronic infectious diseases, postponing treatment.
