Acrodermatitis chronica atrophicans overview
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Anahita Deylamsalehi, M.D.[2] Raviteja Guddeti, M.B.B.S. [3]
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Overview
Acrodermatitis chronica atrophicans is a skin rash indicative of the third or late stage of European Lyme borreliosis. It is a dermatological condition that takes a chronically progressive course and finally leads to a widespread atrophy of the skin. Involvement of the peripheral nervous system is often observed, specifically polyneuropathy.
Historical Perspective
First record of acrodermatitis chronica atrophicans was made in 1883 in Breslau, Germany, where a physician named Alfred Buchwald first delineated it. Later in 1902 Herxheimer and Hartmann described it as a "tissue paper" like cutaneous atrophy and there were first physicians that came up with acrodermatitis chronica atrophicans's name. They described the biphasic manner of this disease by demonstrating both inflammatory and atrophic phases of it. In 1950s the possibility of human to human transmission was discussed.For the first time in 1984, borrelia was discovered as the responsible etiology of acrodermatitis chronica atrophicans.
Pathophysiology
Acrodermatitis chronica atrophicans is one of the tertiary presentations of European lyme borreliosis with Borrelia afzelii known as the most predominant responsible microorganism. Nevertheless other borrelia species such as borrelia garinii and borrelia burgdorferi (B. burgdorferi sensu lato) have been also detected in acrodermatitis chronica atrophicans patients. Transmission of this infection probably occur via ixodes tick (such as Ixodes ricinus), mosquito and horsefly bite. These vectors themselves get infected by feeding on an infected animal reservoir. Development of various symptoms in this disease is a result of chronic T cell mediated reaction of immune system against borrelia. This immune reaction leads to infiltration of CD3+ and CD4+ cells in the dermis. Borrelia is capable of attaching to the extracellular matrix proteins (such as glycosaminoglycan, fibronectin and decorin proteoglycan) which eventually leads to metalloproteases activation and extracellular matrix degradation. Pro-inflammatory cytokines, such as tumor necrosis factor alpha and interleukin-4, have been detected in skin biopsies. There is no known gene responsible in pathophysiology of acrodermatitis chronica atrophicans disease. Some conditions such as lymphocytic meningoradiculitis, lichen sclerosus et atrophicus, morphea and other tick borne diseases have been associated with acrodermatitis chronica atrophicans. Thinning of skin, visible veins, swelling and wrinkles are some of the features can be noticed on gross pathology. Light and electron microscopic study of the skin biopsy shows degeneration of the elastica and collagen fibers. Thinning of dermis and epidermis, pigmented stratum germinativum, dermal blood vessels dilation and perivascular plasma cell infiltration are some of the findings on microscopic pathology.
Causes
This progressive skin disorder is due to the effect of chronic infection with the spirochete borrelia afzelii, which is the predominant cause of acrodermatitis chronica atrophicans. However borrelia afzelii is not the exclusive etiologic agent of acrodermatitis chronica atrophicans and other microorganisms such as borrelia garinii and borrelia burgdorferi have also been detected.
Differentiating Acrodermatitis Chronica Atrophicans from Other Diseases
Acrodermatitis chronica atrophicans must be differentiated from chronic venous insufficiency, chronic arterial insufficiency, superficial thrombophlebitis, frostbite, morphea, erysipelas, acrocyanosis and granuloma annulare.
Epidemiology and Demographics
Acrodermatitis chronica atrophicans is a rare disease. The prevalence of acrodermatitis chronica atrophicans is estimated to include 10% of cases with lyme disease in Europe. The incidence of acrodermatitis chronica atrophicans increases with age and commonly affects individuals in range of 40 to 70 years old with a median of 64 years old. However there are few case reports on children who are diagnosed with acrodermatitis chronica atrophicans. Acrodermatitis chronica atrophicans affects women more than men. The majority of acrodermatitis chronica atrophicans cases are reported in northern, central and eastern Europe (most commonly in countries bordering the Baltic Sea). Lately few cases of acrodermatitis chronica atrophicans have been reported in the United States and Canada.
Risk Factors
Common risk factors in the development of acrodermatitis chronica atrophicans include tick exposure, female gender and residents of northern, central and eastern Europe.