It may be difficult to differentiate these lymphomas from lymphomatoid papulosis, which often has an anaplastic histology, but which clinically has a benign disease course with spontaneous remissions. Good dialogue between the pathologist and clinician is crucial.
Primary cutaneous anaplastic large cell lymphoma is usually confined to one lesion and has a very good prognosis.
Primary cutaneous anaplastic large cell lymphoma is treated with local surgery and/or local radiation therapy without any form of systemic treatment.
Only more disseminated forms are treated with chemotherapy (CHOP).