Localized scleroderma associated with Borrelia burgdorferi infection : clinical, histologic, and immunohistochemical observations

Recent reports have implicated Borrelia burgdorferi infection as a possible cause of localized scleroderma (LS). Our purpose was to describe the clinical, histologic, and immunopathologic features of patients with LS who had serum antibodies to B. burgdorferi. Ten patients were examined clinically and by routine microscopy. Biopsy specimens from seven patients were studied immunohistochemically with monoclonal antibodies. The proliferative response of peripheral blood mononuclear cells to B. burgdorferi was investigated in seven patients by lymphocyte proliferation assay. Seven patients had plaque-type morphea, and three patients had linear scleroderma. Two patients had a history of previous erythema migrans. One patient had coexistent acrodermatitis chronica atrophicans, and in two patients lichen sclerosus et atrophicus was observed. Histologically, a prominent inflammatory phase with sclerosis of the connective tissue was shown in all patients. Immunohistochemical studies revealed that the inflammatory infiltrates consisted of both B and T lymphocytes, predominantly of the CD4+ subset. All 10 patients had strongly elevated serum antibodies to B. burgdorferi. Patients with LS showed significantly elevated lymphoproliferative responses to B. burgdorferi when compared with healthy control subjects. Our findings suggest that some cases of LS are linked to Borrelia infection.