LymeNet Europeinformation and discussion about Lyme disease
Lorima wrote: I did see two unusual and noteworthy things today: a black swan, and a classic ACA case. The swan was at the zoo, and the ACA case is here in southeastern Pennsylvania, in the USA. Where there is said to be no ACA. Is there anything in the dogma about this disease I can believe? Probably not. I get burned every time.
I'll take some pictures of the ACA, if my relative doesn't mind. I wonder what kind of biopsy is needed to culture, or view.
Acrodermatitis chronica atrophicans (ACA) is the third or late stage of European Lyme borreliosis.[1, 2] This unusual progressive fibrosing skin process is caused by an ongoing active infection with Borrelia afzelii. First delineated in 1883, it was described in 1902 as a tissue paper–like cutaneous atrophy.
ACA is evident on the extremities, particularly on the extensor surfaces. It begins with an inflammatory stage characterized by bluish red discoloration and cutaneous swelling and concludes several months or years later with an atrophic phase. Sclerotic skin plaques may also develop. Physicians should use serologic and histologic examination to confirm this diagnosis.
The choice of treatment for ACA depends on the coexistence of other signs or symptoms of Lyme borreliosis. Appropriate consultations (ie, a neurologist, ophthalmologist, rheumatologist, or cardiologist) should be sought if extracutaneous signs and symptoms are present. ACA patients without concurrent extracutaneous disease do not require hospitalization.
Genetic profiles of strains of B burgdorferi sensu lato derived from patients with acrodermatitis chronica atrophicans (ACA) often show the highly conserved MLa1 pattern characteristic of B afzelii; however, they may demonstrate the large restriction fragment patterns typical of B garinii.
The diagnosis of ACA should be based on the following:
• Careful evaluation of the history, including epidemiologic data and symptoms of early and late infection
• A detailed physical examination
• Specific serologic tests results and the histopathologic picture of skin biopsy specimens
A negative history of exposure to ticks should not exclude the diagnosis. B afzelii can be identified in skin lesions by means of polymerase chain reaction (PCR) testing, but both cultures and direct spirochetal stains are usually negative.
Because ACA is often confused with vascular conditions, serologic proof of the diagnosis and histologic verification are considered obligatory. PCR testing for Borrelia -specific DNA (rather than culture of the spirochete) is sometimes a necessary adjunctive measure. Borrelia has been only rarely isolated from the blood culture in these patients. For example, in one study, the organism was isolated from blood in only 3 (1.5%) of 200 ACA patients.
The most important histologic findings in ACA are the presence of telangiectasias and the presence of cellular infiltrates of lymphocytes with admixed plasma cells in the absence of any other explanation for the plasma cells (eg, syphilis or myeloma). Although these changes are not diagnostic, they are highly suggestive (see the images below).
[Some visuals for pathologists and possibly other medical professionals]
In its atrophic stage, ACA demonstrates striking epidermal atrophy, a normal zone just below the epidermis, dilated blood vessels, and a lymphocytic–plasma cell infiltrate within the upper dermis. Plasma cell–rich infiltrates within a sclerotic dermis should suggest the possibility of ACA. Neural lymphocytic infiltration may be evident. Leukocytoclastic vasculitis or vessel occlusion may be seen in some cases. The subcutis is involved in a large percentage of patients.
Warthin-Starry stains may show spirochetes in some cutaneous biopsy specimens, though this often is not the case. Immunohistochemical studies show few B cells despite a substantial number of plasma cells.
Acrodermatitis chronica atrophicans. ACA is the cutaneous manifestation of late-stage Lyme borreliosis in Europe. Very few cases of ACA have been reported in the United States, most likely explained by the near-exclusive causation of ACA by B. afzelii—a borrelial genospecies that is not present in North America.
The differential diagnoses of ACA include deep vein thrombosis, superficial thrombophlebitis, arterial occlusive vascular disease, acrocyanosis, livedo reticularis, lymph-edema, pernio (chilblains), erysipelas, bursitis/arthritis, and morphea.
Acrodermatitis chronica atrophicans (ACA) is a rare tertiary manifestation of Lyme borreliosis, manifesting as inflammatory and atrophic lesions on acral skin. Although ACA rarely has been reported in the United States, it may be seen in approximately 10% of European cases of Lyme borreliosis, most commonly associated with the genospecies Borrelia afzelii. We report a presumptive case of ACA involving an American woman from Pennsylvania with convincing clinical, histopathologic, and serologic findings. We also provide an overview of the history, epidemiology, pathogenesis, clinical and histopathologic presentation, and treatment of ACA.
Newer and more sophisticated diagnostic techniques are continually being developed, making traditional microscopic evaluation of tissue sections appear rather old fashioned. Even the era of immunohistochemistry, in situ hybridization, and computer-assisted image analysis is being supplanted, for example, by the rise of molecular diagnostics using tissue microarrays or in vivo methods such as confocal scanning laser microscopy.1 It is generally recognized that polymerase chain reaction (PCR) techniques can detect evidence of infection or neoplasia even where none is evident by microscopic methods.2 - 3 Therefore, it is encouraging to those of us who are life-long microscopists to learn of a new technique that is largely founded on classic histopathologic skills. Such a technique is focus-floating microscopy. In this issue of the Archives, Eisendle et al4 demonstrate the successful use of this method in identifying Borrelia species in tissue sections from patients with lichen sclerosus.
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