Lymphomatoid papulosis (LyP) is defined as a recurrent self-healing papulonodular eruption with the histological features of a (CD30+) cutaneous T-cell lymphoma. The atypical cells usually have a CD3+/-, CD4+/-, CD8-, CD30+, CD56- T-cell phenotype. We report an unusual case of LyP, in which the atypical cells expressed a CD3-, CD4-, CD8-, CD30+, CD56+ phenotype. Detailed phenotypic and genotypic analysis confirmed that these cells had a natural killer (NK)-cell phenotype. Lymphomas with an NK-cell phenotype usually have a poor prognosis. However, the waxing and waning of papular lesions for more than 20 years and the excellent response to low-dose oral methotrexate in this patient suggest similar clinical behaviour to LyP cases with a T-cell phenotype.
Tumour necrosis factor-alpha (TNF-α) antagonists, such as etanercept, infliximab and adalimumab, are used increasingly in the treatment of chronic inflammatory diseases, such as rheumatoid arthritis (RA), ankylosing spondylitis, psoriatic arthritis, moderate to severe psoria-sis and Crohn’s disease (1). In recent years there has been an increasing number of reports on malignant lymphomas developing in patients using TNF-α blocking agents, mostly infliximab or etanercept. Most lymphomas deve-loping in patients using anti-TNF-α therapy are B-cell lymphomas (2). Reports on T-cell lymphomas as a result of this treatment, especially those primarily involving the skin, are rare (3–11). We report here an unusual case of cutaneous γδ T-cell lymphoma (CGD-TCL) in a patient with RA receiving anti-TNF-α treatment.CASE REPORT
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