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[Livedo racemosa: an unusual late manifestation of borreliosis?].

Classic variants of cutaneous borreliosis are erythema chronicum migrans (ECM), lymphadenosis benigna cutis (LBC) and acrodermatitis chronica atrophicans (ACA). Other dermatoses have been reported in the literature as possibly linked to borreliosis. A 59-year old female patient was seen in the late phases of cutaneous borreliosis with histologically confirmed ACA. In addition, prominent livedo racemosa was seen on the legs, also showing tissue changes similar to those of ACA. Borrelia burgdorferi infection was serologically confirmed by the presence of anti-IgM and anti-IgG antibodies. The clinical spectrum of late cutaneous borreliosis should be enlarged to include livedo racemosa.

Acrodermatitis↗

The many faces and phases of borreliosis II.

Borrelia burgdorferi, the etiologic agent of Lyme disease, has also been associated with other cutaneous conditions. Acrodermatitis chronica atrophicans and lymphadenosis benigna cutis are also caused by B. burgdorferi. Recent evidence links some cases of progressive facial hemiatrophy of Parry-Romberg, benign lymphocytic infiltrate of the skin (Jessner-Kanof), lichen sclerosus et atrophicus, morphea, and Shulman syndrome with borreliae. This article reviews the manifestations of the diseases definitely linked to borreliosis and the evidence linking borreliae to progressive facial hemiatrophy, benign lymphocytic infiltrate, lichen sclerosus et atrophicus, morphea, and Shulman syndrome.

Acrodermatitis↗

Spirochetes in atrophic skin lesions accompanied by minimal host response in a child with Lyme disease.

Acrodermatitis chronica atrophicans, which has rarely been observed in the United States, is a late skin manifestation of Lyme borreliosis. A 12-year-old girl who spent summers on Cape Cod presented with a 2-year history of hyperpigmentation and atrophy of the skin on the hands, wrists, and ankles. The skin biopsy specimen of an affected area showed mild dermal fibrosis, a few inflammatory cells, and spirochetes morphologically compatible with Borrelia burgdorferi. An IgG antibody response to B. burgdorferi could be elicited by immunoblotting, but not by enzyme-linked immunosorbent assay. We conclude that this patient had chronic Lyme borreliosis manifested only by indolent infection of the skin.

Acrodermatitis↗

Evidence for the involvement of different genospecies of Borrelia in the clinical outcome of Lyme disease in Belgium.

In addition ot Borrelia burgdorferi, recognized as the aetiological agent of Lyme disease, at least two separate genospecies have recently been described. A relationship between infection by strains belonging to different genospecies and clinical outcome has been suspected. In this paper, 9 cases of Lyme arthritis were attributed to infection by B. burgdorferi sensu stricto, 18 cases of neuroborreliosis to B. garinii and one case of acrodermatitis chronica atrophicans to a strain of B. afzelii. These conclusions were based on the preferential reactivity of sera with antigens of given strains in Western blots and on residual reactivity after absorption of sera with antigens of representative strains. No conclusion could be reached concerning sera of 10 patients with erythema migrans.

Acrodermatitis↗

Association between different clinical manifestations of Lyme disease and different species of Borrelia burgdorferi sensu lato.

Borrelia burgdorferi sensu lato, the aetiological agent of Lyme disease, has been subdivided into three species: B. burgdorferi sensu stricto, B. garinii and B. afzelii. We and other authors have hypothesized an association between the three species of B. burgdorferi sensu lato and some of the different clinical manifestations of Lyme disease. In order to demonstrate this hypothesis, we analysed twenty-nine isolates cultured from patients with different symptoms. The method used was multilocus enzyme electrophoresis: twelve genetic loci were characterized on the basis of the electrophoretic mobility of their products, and twenty-eight distinctive allele profiles (electrophoretic types) were distinguished, among which mean genetic diversity per locus was 0.649. Cluster analysis of a matrix of genetic distances between paired electrophoretic types revealed three primary divisions separated at genetic distances greater than 0.7 and corresponding to the three species of B. burgdorferi sensu lato. Ten strains obtained from skin of patients with erythema chronicum migrans (the primary stage of the disease) were assigned to the three different species. All the six strains isolated from patients with acrodermatitis chronica atrophicans were of the species B. afzelii, which was not found to be associated with another chronic manifestation of Lyme disease. Arthritis was caused prevalently by B. burgdorferi sensu stricto, and neuroborreliosis by B. burgdorferi sensu stricto and B. garinii. In conclusion, our results confirm the association between some of the different chronic manifestations of the disease and the species of B. burgdorferi sensu lato.

Acrodermatitis↗

Zinc uptake into fibroblasts is inhibited by probenecid.

Cellular zinc transport has not been fully characterized. The role of an anion carrier was investigated by treating normal human fibroblasts, and those carrying a mutation which affects zinc transport, acrodermatitis enteropathica (AE), with the anion carrier inhibitor, probenecid. Zinc uptake (2, 10, or 20 micromol 1(-1) 65zinc) was determined during initial rates of uptake (15 min) following treatment with 0, 10 or 20 mmol 1(-1) probenecid. Probenecid stimulated extracellular zinc binding in normal and AE fibroblasts. Probenecid inhibited the internalization of zinc in normal, but not AE, fibroblasts. Normal fibroblasts exhibited an apparent Km which was reduced by 53% and 44% in the 10 and 20 mmol 1(-1) probenecid treated cells. The Vmax was also reduced in the normal fibroblasts by 51% and 50% in the 10 and 20 mmol 1(-1) probenecid treated cells. The results suggest that a probenecid-sensitive anion carrier is involved in the internalization of zinc in human fibroblasts. The lack of an effect of probenecid on the internalization of zinc in the AE fibroblasts suggests that the mutation involves a probenecid-sensitive anion transport system, and that there may be a secondary mechanism for zinc transport in these cells.

Acrodermatitis↗

Ultrastructural differences among spirochetes isolated from patients with Lyme disease and related disorders, and from Ixodes ricinus.

Previous studies on cells of strains B31 isolated in the U.S.A. from Ixodes dammini and strain G25 isolated in Sweden from Ixodes ricinus, showed that their ultrastructure was similar, but not identical. For this reason the studies were extended to spirochetes isolated directly from patients with Lyme disease and related disorders. Included in the present study were three strains isolated from skin, blood and spinal fluid, respectively, from patients with Lyme disease, two strains from patients with erythema chronicum migrans and one strain from a patient with acrodermatitis chronica atrophicans. Three additional strains isolated in Sweden from Ixodes ricinus were also studied. All spirochetes were examined after negative straining with 1% ammonium molybdate. The cells of each individual strain were identical except for one strain isolated from a tick. This isolate was found to consist of two morphologically different spirochetes. Comparison of morphological features of cells from various isolates revealed certain differences. The cells of the different strains could be divided into at least four groups for which cell size and shape as well as number of flagella varied. By morphological criteria, all cells were found to belong to the genus Borrelia.

Acrodermatitis↗

Clinical features of early erythema migrans disease and related disorders.

104 patients with erythema migrans disease (EMD), 7 patients with borrelia lymphocytoma (BL), and 21 patients with acrodermatitis chronica atrophicans (ACA) were prospectively followed for a median of 20, 14, and 12 months, respectively. 99 patients with EMD and 6 with BL were treated with antibiotics early for their illness. 72 patients with EMD had 1 to 10 constitutional symptoms besides the erythema migrans, 32 had erythema migrans alone, and a child with BL had urticaria. Out of 39 patients with EMD, 23 acquired arthralgia, 18 signs and symptoms consistent with neurologic manifestations and 8 with cardiac involvement before or after therapy. 4 patients with EMD and 1 with BL had up to 10 multiple erythema migrans lesions. 3 patients with EMD experienced a reinfection and 1 with ACA a relapse. Several patients with ACA developed signs and symptoms consistent with neurologic, cardiac and joint involvement, and 2 had a history of EMD. Elevated antibody titers against Borrelia burgdorferi were present in 48% of 69 patients with EMD, 5 with BL and 11 with ACA, and in 93% of another group with EMD, 2 with BL and 10 with ACA, when tested against other borreliae. Increased values of the ESR, IgG, IgA and IgM were found in more patients with ACA than with EMD. The median of IgA and IgM was significantly higher in ACA than in EMD. Borreliae were found in brain and liver of a newborn. Early EMD appears to be quite similar to early Lyme disease.

Acrodermatitis↗

Joint and bone involvement in Swedish patients with Ixodes ricinus-borne Borrelia infection.

The presence of signs of joint involvement was investigated in 231 patients with erythema chronicum migrans Afzelius (ECMA), in 50 patients with acrodermatitis chronica atrophicans (ACA), in four patients with spirochete-induced facial palsy and in one patient with pericarditis and serologic evidence of Borrelia spirochetal infection. Only one of 16 untreated patients with ECMA developed arthritis. The patient with pericarditis had suffered from arthritis for 2 months when the cardiac symptoms developed. Thirteen of the patients with ACA had luxations/subluxations of small joints in the hands or feet and/or arthritis in large joints. In four of the patients with ACA, periosteal thickening of bones was found. The results indicate that joint abnormalities are not uncommon in patients with ACA. However, arthritis during the first year of tick-borne spirochetal infection was less common in Sweden than has been reported among patients with Lyme disease in the United States.

Acrodermatitis↗

Antibiotic therapy in early erythema migrans disease and related disorders.

Between December 1978 and July 1985, we used various antibiotics for the treatment of 97 adult patients with early erythema migrans disease (EMD). Six patients with borrelial lymphocytoma (BL) and 20 with acrodermatitis chronica atrophicans (ACA) were treated similarly. Follow-up was for a median of 20, 14, and 12 months, respectively. The erythema migrans and all associated symptoms resolved within a median of 3 weeks (0.5-18.4), BL within 7 weeks (4-16), and ACA partly or completely within several months. A Jarisch-Herxheimer (-like) reaction was observed in 8 patients with EMD. Fourteen patients with EMD and one with ACA developed an exacerbation of symptoms or new manifestations between the 2nd and 20th day, and 28 patients with EMD and one with ACA continued to have or acquired various symptoms greater than or equal to 3 weeks after initiation of therapy. Arthralgia, neurologic and constitutional symptoms, and in one instance a slight pulmonary interstitial edema developed in EMD. More severe initial illness was a risk factor for the development of later symptoms in EMD. Retreatment was more often necessary in ACA than in EMD. A patient with ACA had a recurrence after 5 1/2 years. IgG antibody titers rose at least fourfold in 5 patients with ACA and in 1 with EMD despite therapy. We tentatively recommend minocycline or high doses of parenteral penicillin for the treatment of these disorders.

Acrodermatitis↗

Erythema chronicum migrans disease in the Federal Republic of Germany.

Epidemiology and clinical presentation of Erythema chronicum migrans disease are not well known yet. During a period of only 19 months, serological and clinical investigation of 2955 patients rendered 1106 cases of infection whose widespread incidence was remarkable: of the 328 administration districts of the FRG, 205 were affected. Accordingly, positive antibodies against Borrelia burgdorferi could be demonstrated in an average of 15.7% of the investigated rural population (2830 persons). Typical clinical signs were encountered in 817 of 1106 infected persons. Erythema (458 cases) and meningopolyneuritis (404 cases) were especially prominent. In comparison to Lyme disease the occurrence of arthritis (63 cases), carditis (13 cases) multiple erythema, recurrence, and central nervous symptoms in meningopolyneuritis (10%) were rare. On the other hand, progressive borrelia encephalomyelitis (45 cases) was surprisingly common. Acrodermatitis chronica atrophicans occurred in 72 cases; lymphadenosis benigna cutis in 5 patients. The variability of this disease is demonstrated by the combined syndromes occurring in only 27% of the cases.

Acrodermatitis↗

Epidemiology of borrelia infections in Austria.

From April 1984 to July 1985 873 cases of Borrelia infections were registered at the Hygiene Institute of the University of Vienna. 2609 serum samples of these patients were investigated for antibodies against B. burgdorferi by means of IFA- and ELISA-tests. Erythema chronicum migrans (ECM) was recognized in 60.9% of patients, neurological abnormalities were recorded in 23.4% of which the majority manifested themselves as polyradiculitis and meningopolyneuritis (MPN). Acrodermatitis chronica atrophicans (ACA) was recognized in 11.5%. A small number of patients suffered from Lymphadenosis cutis benigna (LCB), arthritis and cardiac abnormalities. Sixty percent of patients were females and 40% males. Infections were found in all age groups ranging from 2-83 years in females and 1-85 years in males. Tick- or insect-bites prior to the onset of illness were reported by 47.2% and 15.6% of patients, respectively. The main vector is the hard tick Ixodes ricinus. Flying insects from the family tabanidae, i.e. Chrysops caecutiens and Haematopota species, must also be considered as transmitters. Antibodies to B. burgdorferi were found in 22.3%, 93.6% and 100% of sera from patients with ECM, MPN and ACA, respectively. Six of 11 patients with LCB and all with arthritis and cardiac abnormalities showed serologic reactivity. Geographically, Borrelia infections are distributed in all states of Austria. The seasonal distribution of cases show a peak in July and August, but the onset of clinical manifestation could be observed throughout the year. These results present Austria as an area where tick- or insect-borne Borrelia infections are very frequent and endemic in all Austrian states.

Acrodermatitis↗

Gianotti-Crosti syndrome associated with hepatitis B surface antigen (subtype adr).

The Gianotti-Crosti syndrome, papular acrodermatitis of childhood (PAC), is an infrequently recognized disorder with distinctive characteristics. At present hepatitis B virus is thought to be an etiologic agent. The disease is very rare in Korea, in spite of the high frequency of hepatitis B surface antigen (HBsAg) in the general population. It is known that subtype ayw of the HBsAg may influence the pathogenesis of PAC, but subtype analysis of HBsAg in these patients disclosed adr. Therefore, our studies reconfirmed that PAC may in addition be associated with subtype adr of HBsAg. We believe that the lower incidence of PAC in Korea as compared with the high incidence of PAC in other parts of the world, such as in the Mediterranean area, may be due to the fact that a higher predisposition to PAC is conferred by subtype ayw of HBsAg.

Acrodermatitis↗

Inborn errors of trace element metabolism.

Genetic disorders of trace element transport are now known in humans, mice, dogs and cattle. Those involving copper have been known longest and are best known clinically. Effects due to copper deficiency are seen in Menkes' disease, in X-linked cutis laxa and in the X-linked series of mottled mutants in the mouse. Copper accumulation is also harmful, causing damage initially to the liver and later to the kidneys and brain in Wilson's disease, in some Bedlington terriers and in toxic milk mice. Zinc deficiency is seen in acrodermatitis enteropathica and in premature babies born to women who seem to secrete milk that is zinc-deficient, as is seen in lethal milk mice. Study of animal mutants, especially mutant mice, is helpful in understanding the human diseases and identification of the basic defects in trace element transport in these diseases is improving knowledge relevant to trace element nutrition.

Acrodermatitis↗

Current aspects of Lyme disease and other Borrelia burgdorferi infections.

Lyme disease, acrodermatitis chronica atrophicans, and borrelial lymphocytoma are caused by species of the spirochete Borrelia burgdorferi. Lyme disease has emerged as the leading vector-borne infectious disease in the United States. This article presents a current review of these entities.

Acrodermatitis↗

[Zinc deficiency in mucoviscidosis].

UNLABELLED: Zinc deficiencies can induce dermatitis in subjects presenting cystic fibrosis. CASE REPORT: A patient, Clement, presented with a digestive form of cystic fibrosis. At four months of age, he presented a dermatitis similar to acrodermatitis enteropathica. Early clinical diagnosis and treatment led to a rapid response to zinc sulfate therapy. DISCUSSION: Less intestinal absorption, malnutrition, and diet are just some of the numerous reasons for the zinc deficiency in this case. Biological support is not necessary to begin the treatment.

Acrodermatitis↗

Longterm survey (7 years) in a population at risk for Lyme borreliosis: what happens to the seropositive individuals?

In 1986, a 26% seroprevalence of IgG- anti-Borrelia burgdorferi antibodies was observed among 950 orienteers and the incidence of new clinical infections was 0.8%. In 1993, a total of 305 seropositive orienteers were reexamined. During that time, 15 cases (4.9%) of definite/probable Lyme disease occurred in this seropositive group (12 skin manifestations and 3 monoarticular joint manifestations). Among the 12 definite cases, 9 showed new clinical infections (7 EM, 1 acrodermatitis chronica atrophicans, 1 arthritis), and 3 were recurrent (2 EM, 1 arthritis). The annual incidence (0.8%) in this seropositive group was identical to the incidence observed among the whole population in 1986. The individual antibody titer decreased slightly but the seroreversion rate was low (7%). Serology was not very helpful in identifying clinical cases and evolutions, and it can be stated, that a positive serology is much more frequent in this risk group than clinical disease.

Acrodermatitis↗

[Late manifestations of Lyme borreliosis].

Month to years after an early local or an early disseminated infection some patients develop late manifestations of lyme borreliosis. Most frequently involved organs are the skin (acrodermatitis chronica atrophicans), joints (Lyme arthritis) and the nervous system. A history of exposure and the clinical picture may suggest Lyme borreliosis, however, confirmation by serological and other tests is needed. Antibiotic treatment during early stages normally prevents development of late manifestations. Late stages persist if not treated. By adequate antimicrobial therapy they are treatable and usually show a good prognosis. Recovery may be delayed, some patients suffer from residual difficulties. Currently there is no accepted case definition for a "post lyme syndrome". The term "chronic Lyme disease" suggests (a never proven) persistent infection by viable bacteria. Repeated and prolonged antibiotic treatments are not indicated.

Acrodermatitis↗