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[Acrodermatitis chronica atrophicans in the course of old Lyme disease].

Lyme borreliosis is chronic spirochetal infection with phasic course. Lyme disease can be recognised as early or late stage of disease. A case report of patient with acrodermatitis chronica atrophicans in the course of chronic (late) Lyme borreliosis is presented. As it is described, the lack of uncognition of infection of B. burgdorferi and typical signs of erythema migrans as well as a lack of proper therapy in this time may be a reason of passing into chronic phase of the disease like ACA and Lyme arthritis.

Acrodermatitis↗

Acrodermatitis chronica atrophicans involving the face. Evidence for Borrelia burgdorferi infection confirmed by DNA amplification.

In a female patient with increasing redness of the hands and face, proteins of Borrelia burgdorferi were detected in a biopsy of the ear by DNA amplification. Although acrodermatitis chronica atrophicans has been documented to be caused by B. burgdorferi, this is the first case with proven spirochetal presence in the skin of the head. After 2 weeks of intravenous therapy with ceftriaxone marked improvement of discoloration of the skin was noted.

Acrodermatitis↗

Acrodermatitis chronica atrophicans Herxheimer can often mimic a peripheral vascular disorder.

During the past few years five patients have been referred to the angiology section at Danderyd Hospital under the diagnosis of chronic venous insufficiency but who were instead suffering from acrodermatitis chronica atrophicans (ACA). The typical case of ACA starts with a limited inflammatory lesion, which is gradually replaced by atrophy and the skin shows a bluish, red discoloration. Late changes may be subluxation of joints in hands or feet and periosteal thickening. Capillary microscopy often reveals a clear picture of atrophy and a prominent, dilated subpapillary venular plexus. If localized to the leg the blood flow of the foot and lower leg may be increased, skin temperature elevated and venous capacity and return augmented. All these variables can consequently mimic venous insufficiency of the leg. ACA is caused by a Borrelia infection and serological testing will always show a significantly elevated titer to Borrelia. The disease is most often easily cured by 2-3 g of penicillin daily for two to three weeks.

Acrodermatitis↗

Serologic studies of erythema chronicum migrans Afzelius and acrodermatitis chronica atrophicans with indirect immunofluorescence and enzyme-linked immunosorbent assays.

To determine whether antibodies to Borrelia spirochetes were present, sera from 88 patients with uncomplicated erythema chronicum migrans Afzelius (ECMA), from 9 patients with ECMA-related extracutaneous complications and from 26 patients with acrodermatitis chronica atrophicans (ACA) were submitted to an enzyme-linked immunosorbent assay (ELISA) and an indirect immunofluorescence (IF) assay. The assays were calculated to be 95% specific. There was good correlation between the IF test with a polyvalent conjugate and IgG ELISA. Of patients with uncomplicated ECMA, 18% were seropositive by IgG ELISA and 11% by IgM ELISA, and 15% showed elevated IF titers. Elevated serum antibody levels of IgG as measured by ELISA and elevated IF titers were found in all patients with extracutaneous complications and in the patients with ACA. Declining IgG titers were observed at follow-up 6-12 months after therapy, but the majority of the patients with ACA were still seropositive.

Acrodermatitis↗

Acrodermatitis chronica atrophicans--a spirochetosis. Clinical and histopathological picture based on 32 patients; course and relationship to erythema chronicum migrans Afzelius.

The recent discovery that spirochetes transmitted by the tick Ixodes ricinus are involved in the etiology of erythema chronicum migrans Afzelius (ECMA), Bannwarth's syndrome, and acrodermatitis chronica atrophicans (ACA) has thrown new light upon these disorders. Thirty-two patients showing clinical and serological evidence of ACA were investigated. Histologically, constant findings in active ACA lesions were telangiectases and a lymphocytic infiltrate with a moderate to rich admixture of plasma cells. Clinically, besides ACA lesions, lichen sclerosus et atrophicus (LSA)-like lesions were found in five patients. Four of these patients displayed a histopathological picture compatible with LSA. These findings suggest a relationship between ACA and LSA. In six patients spontaneous healing of ECMA was followed by ACA lesions after a latency period of 1-8 years. Six patients reported histories of cranial nerve involvement. Radiography revealed subluxation of joints in hands or feet in six patients, and periosteal thickening in another three patients. The results indicate that ACA may be a late manifestation of infection with the same spirochete that causes ECMA and Bannwarth's syndrome. If untreated, the infection may continue for many years and result in irreversible degenerative lesions.

Acrodermatitis↗

Antibiotic therapy of early European Lyme borreliosis and acrodermatitis chronica atrophicans.

In a study on 121 consecutive patients with erythema migrans, 65 patients obtained oral penicillin, 36 tetracyclines, and 20 amoxicillin-clavulanic-acid. Follow-up was carried out for a median of 29, 17, and 7 months, respectively. In another limited trial on 29 patients with acrodermatitis chronica atrophicans (ACA), 14 patients received oral penicillin, 9 parenteral penicillin, and 6 tetracyclines. There was no statistically significant difference among treatment groups in both therapeutic trials, with the exception of different follow-ups due to the nonrandomized study design and different occurrence of the Jarisch-Herxheimer reaction in patients with erythema migrans. Later extracutaneous manifestations developed in 27% of the patients with erythema migrans and in 47% of the patients with ACA despite antibiotic therapy. We could not prove the superiority of any antibiotic tested in either early or late European Lyme borreliosis.

Acrodermatitis↗

[Etiology of acrodermatitis chronica atrophicans and erythema chronicum migrans].

In Lyme's disease (LD) as well as in the European form of erythema chronicum migrans (ECM), the etiologic agents are spirochetes. As fas as we know by now, these microbes are closely related but not identical. Consequently, LD and ECM should be regarded as closely related but not as identical diseases. The sera of our 21 patients suffering from acrodermatitis chronica atrophicans (ACA) contained elevated antibody titers directed against the etiological agent found in ECM. These findings strongly suggest that ACA is also induced by spirochetes-possibly by the same microbes found in ECM.

Acrodermatitis↗

Serodiagnosis of erythema migrans and acrodermatitis chronica atrophicans by the Borrelia burgdorferi flagellum enzyme-linked immunosorbent assay.

The diagnostic performance of an enzyme-linked immunosorbent assay (ELISA) using purified Borrelia burgdorferi flagella as test antigen was compared with that of a B. burgdorferi sonic extract ELISA. We tested sera from 200 healthy controls, 107 patients with erythema migrans (EM), 50 patients with acrodermatitis chronica atrophicans (ACA), and 98 patients with various dermatological disorders without clinical evidence of active Lyme borreliosis. The flagellum ELISA was significantly more sensitive than the sonic extract ELISA. With sera from patients with EM, the diagnostic sensitivity for immunoglobulin G (IgG) antibody detection increased from 11.2 to 35.5% (P less than 0.001) and for IgM antibody detection it increased from 16.6 to 44.8% (P less than 0.001). In the flagellum ELISA, the number of positive tests increased significantly (P less than 0.005) when the duration of EM exceeded 1 month, but still only about 50% of patients with longstanding (1 to 12 months) untreated EM were IgG seropositive. Concomitant general symptoms did not affect the antibody level, whereas patients with multiple erythema were more frequently seropositive. All sera from patients with EM which were positive in the sonic extract ELISA were also positive in the flagellum ELISA. Not only did the overall number of positive tests increase, but the flagellum ELISA yielded a significantly better quantitative discrimination between seropositive patients and controls (P less than 0.002). IgG antibodies to the B. burgdorferi flagellum were found in all sera from patients with ACA, indicating persistence of an antiflagellum immune response in late stages of Lyme borreliosis. IgM reactivity in sera from patients with ACA was shown to be unspecific and the result IgM rheumatoid factor. A rheumatoid factor was detected in sera from 32% of patients with ACA, compared with 7.5% of patients with EM. The improved diagnostic performance, the ease of standardization of the flagellum antigen, and the lack of strain variation make the B. burgdorferi flagellum a needed reference antigen for growing routine serology in Lyme borreliosis.

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