Erythema chronicum migrans Afzelius and acrodermatitis chronica atrophicans. Early and late manifestations of Ixodes ricinus-borne Borrelia spirochetes.

In the present thesis consecutive patients, 231 with ECMA, 41 with ACA and 20 with facial palsy (Bell's palsy), have been studied. It has been shown that spirochetes, transmitted by the tick Ixodes ricinus, are involved in the etiology of ECMA and ACA. This has been shown through the isolation and cultivation of spirochetes from ticks and from the skin of patients with ECMA and ACA. The spirochetes have morphological characteristics similar to those of the genus Borrelia. No antigenic differences have been found between ECMA and ACA strains by the use of four different monoclonal antibodies against Borrelia burgdorferi. In serological studies, with the indirect IF test and with the ELISA, elevated antibody titers against these spirochetes were found in sera from patients with ECMA and ACA. Significantly increasing titers were found in sera from patients who developed extracutaneous complications and decreasing titers as a response to therapy. The serological tests are of good diagnostic help for patients with ACA and in many of the patients with ECMA-related extracutaneous complications, but in the present study only 15-28% of the patients with uncomplicated ECMA were seropositive. The study has shown that a tick bite and/or an untreated ECMA may be followed by symptoms from the nervous system (facial palsy, meningoradiculitis), the joints and from the heart as well. It has also been shown that ACA may sometimes be preceded by an untreated ECMA and that manifestations from the nervous system and/or the joints may precede or accompany ACA. There may be a long period of latency (several years) between a spontaneously healing ECMA and the development of ACA lesions. In ACA patients with abnormalities of joints or bones the concordance in site of the cutaneous involvement and changes in the underlying joints or bones may in these cases be consistent with a progressive localized spirochetal infection. Lichen sclerosus et atrophicus-like lesions found in patients with ACA indicate that a Borrelia infection may result in lichen sclerosus et atrophicus-like reactions. The recognition of ACA may be difficult and a combination of clinical, histopathological and serological findings may be necessary to secure the diagnosis. Clinical differences between ECMA-related disorders in Sweden an Lyme disease in the United States have been found and there may also be antigenic differences between the spirochetes involved.(ABSTRACT TRUNCATED AT 400 WORDS)