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SummaryBackground The long-term prognosis of patients treated for erythema migrans has only rarely been assessed. Objectives To evaluate the clinical characteristics and long-term prognosis of patients treated for erythema migrans in the region of Alsace, France. Methods In a prospective study, 56 consecutive patients presenting with erythema migrans at the Strasbourg University Hospital between 1995 and 1999 were examined and a Borrelia burgdorferi enzyme immunoassay was performed. Patients were treated with tetracyclines or amoxycillin. Patients were re-examined 6 weeks later and a telephone interview was performed in summer 2000 to evaluate the long-term outcome. Results There were 25 women and 31 men of mean age 49 years presenting with single (n = 54) or multiple (n = 2) erythema migrans lesions. At the time of diagnosis, 30% of the patients had systemic signs, myalgias or arthralgias and only 36% of 50 patients were seroreactive against B. burgdorferi. None of the 51 patients evaluated at 6 weeks and none of the 37 patients interviewed after a median delay of 3 years had developed complications attributable to Lyme borreliosis. Conclusions The prognosis of patients treated for Lyme borreliosis in this part of France is excellent. Therefore, a complete clinical examination is sufficient as an initial evaluation and long-term follow-up is not necessary.

In Europe, Borrelia burgdorferi sensu stricto, Borrelia afzelii and Borrelia garinii are known as pathogens among the Borrelia burgdorferi sensu lato group. Since it is not yet known which Borrelia are responsible for Lyme borreliosis in France, the objective of this study was to identify the species of Borrelia responsible for erythema migrans in the region of Alsace, France.Eighteen patients with erythema migrans (EM) of more than 5 cm of diameter were included in this prospective study. All patients were investigated at the Strasbourg University Hospital. Patients were biopsied on the active border of their lesion. Cutaneous biopsies of the active border of the lesion were cultivated in BSK-H medium (Sigma) and analysed in vitro by PCR after 8 weeks of culture, using flagellin consensus sequences which are present in all species of Borrelia burgdorferi sensu lato group as primers. Species-specific oligotyping was used for species identification.Among the 18 patients biopsied, 7 had evidence of borrelia infection revealed by culture and/or PCR. Borrelia afzelii was detected in 4 patients and Borrelia garinii in three.These preliminary results appear to confirm that Borrelia afzelii and Borrelia garinii are the predominant borrelial species in EM lesions in our geographic area, as in other European countries.

Hereditary progressive mucinous histiocytosis is an uncommon condition which to date has only been observed in women. The disease is a non-Langerhans histiocytosis with a stereotypic clinical presentation limited to the skin. The clinical, histological and genetic features of this disease differ considerably from other types of histiocytoses and overload diseases. We report the first case observed in France.A 49-year-old woman consulted for asymptomatic papules on the dorsal aspect of the hands known since childhood. The lesions had progressively spread to other regions of the body. The woman's mother and two sisters had the same condition but her two sons and her brother and nephews did not. Standard semi-thin slices and electron microscope histology demonstrated dense cellular infiltration of the reticular dermis with interstitial mucinous overload, voluminous histiocytes with characteristic toluidine blue positive granulations, and a cytoplasm rich in myelin and Zebra bodies respectively. The observed clinical, histological and genetic features were characteristic of progressive hereditary progressive mucinous histiocytosis.The pedigree in our case and in those reported in the literature suggest a dominant hereditary condition. The fact that only female cases have been reported to date would suggest X-linked transmission with early death of male fetuses. An autosomal dominant transmission limited to woman as a result of mitochondrial heredity or hormone-related phenomena cannot be ruled out. The progressive extension with age without a tendency for spontaneous regression and the electron microscopy aspect suggest a phospholipid deposit disorder similar to that observed in lysosomial diseases. However, primary macrophage proliferation in response to an unknown stimulus cannot be excluded.