Positive serology for Lyme disease Borrelias in primary cutaneous B-cell lymphoma: A study in 22 patients; Is it a fortuitous finding?

Background: The historical association of acrodermatitis chronica atrophica ns (ACA), now known to be a late manifestation of Lyme disease caused by Bo rrelia afzelii, with cutaneous lymphoma, and several small series of PCBCL with positive Lyme disease borrelial serology initiated a study of this ass ociation. Material and methods: In the last 9 years, 30 patients with PCBCL have been observed and followed, 22 of them were tested for borrelial serology. The control group consisted of 85 patients with NHL (10 cutaneous T-cell, 25 ex tranodal B-cell non-PCBCL, 50 nodal B-cell), 30 patients with breast cancer and 60 blood donors. The screening tests were two different ELISA tests fo r B. burgdorferi sensu late and sensu stricto, and reactive sera were furth er tested with the ELISA test for B. garinii, a Western blot (WB) test for Swiss Borrelia strains and a WE test for Bavarian Borrelia strains, since a n immunoblot made with local strains was not available. Studies with a diff erential WE test for B. burgdorferi sensu stricto, B. garinii and B. afzeli i was performed afterwards. as well as serological studies ruling out cross -reactions with Leptospiras and Treponema. Results: Fifteen of 22 patients with PCBCL were positive on the screening t ests, three of them falsely. Thus, the incidence of positive borrelial sero logy was 12/22 (55 per cent) in the PCBCL group. No positives were detected in the cutaneous T-cell lymphoma group: 2/25 patients (8 per cent) were po sitive in the extranodal B-cell NHL group (the localizations being vestibul um nasi and oral cavity), 2/50 (4 per cent) were positive in the nodal B-ce ll NHL group, 2/30 (7 per cent) in the breast cancer group and 2/60 (3 per cent) in the blood donor group. The cumulative incidence in the control gro ups was 8/175 (4,6 per cent). The incidence was significantly higher in PCB CL patients as compared to each of the control groups, p value ranging from 0.004 to <0.0001. Two positive patients had ACA, one arthritis. Borrelia a fzelii was most often implied for positive serology in the differential WE. No cross-reactions with Treponema and the Leptospiras were documented. Conclusion: In conclusion there appears to be a clustering of positive sero logy for Lyme disease Borrelias in PCBCL patients possibly related to an et hiopathogenic relationship. Mechanisms of Borrelia escape from immunosurvei llance mechanisms, persistence of both their mitogenic and antigenic stimul i for B-cells, and SALT formation may be involved in the pathogenesis of a subset of PCBCL. Copyright (C) 1999 John Wiley & Sons, Ltd.