Acrodermatitis chronica atrophicans overview

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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

Historical Perspective

Pathophysiology

Causes

Differentiating Acrodermatitis chronica atrophicans from other Diseases

Epidemiology and Demographics

Risk Factors

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms

Physical Examination

Laboratory Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

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Overview

First record of acrodermatitis chronica atrophicans was made in 1883 in Breslau, Germany, where a physician named Alfred Buchwald first delineated it. Acrodermatitis chronica atrophicans is one of the tertiary presentations of European lyme borreliosis with Borrelia afzelii known as the most predominant responsible microorganism. 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. Immune reaction against borrelia leads to infiltration of CD3+ and CD4+ cells in the dermis. 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. Acrodermatitis chronica atrophicans must be differentiated from chronic venous insufficiency, chronic arterial insufficiency, superficial thrombophlebitis, frostbite, morphea, and granuloma annulare. 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. Acrodermatitis chronica atrophicans affects women more than men and the majority of acrodermatitis chronica atrophicans cases are reported in northern, central and eastern Europe. Common risk factors in the development of acrodermatitis chronica atrophicans include tick exposure, female gender and residents of northern, central and eastern Europe. The course of acrodermatitis chronica atrophicans is chronic and could lasts for several years and it can progress slowly overtime. In first phase (the inflammatory phase) skin changes appear as blue and red discoloration with boggy infiltration. These inflammatory skin lesions can become atrophic without treatment (atrophic phase). Superimposed bacterial infection, sclerotic skin changes, malignancies, arthropathy and peripheral neuropathy are some of the common complications of acrodermatitis chronica atrophicansis. The general pognosis is good with proper and rapid treatment in acute inflammatory stage of acrodermatitis chronica atrophicans, nevertheless late treatment can cause some irreversible changes. Skin examination of acrodermatitis chronica atrophicans's patients include blue, red or brown discoloration, hypopigmentation, indurated plaques and wrinkles. High anti-spirochetal antibody levels (such as IgG, IgM and IgA) has been detected at indirect immunofluorescence and enzyme linked immunosorbent assay (ELISA). Antibiotic therapy is recommended in patients with acrodermatitis chronica atrophicans. Up to four weeks treatment with antibiotics such as amoxicillin, doxycycline, ceftriaxone, cefotaxime and penicillin G has been recommended for acrodermatitis chronica atrophicans's treatment. Since transmission of borrelia infection occurs by ticks, mosquitos and horse flies bites, primary prevention could be achieved by bite avoidance.

Historical Perspective

Pathophysiology

Causes

Differentiating Acrodermatitis Chronica Atrophicans from Other Diseases

Epidemiology and Demographics

Risk Factors

Natural History, Complications, and Prognosis

Diagnosis

History and Symptoms

History of tick bite, erythema migrans or other symptoms of lyme disease, and rheumatological symptoms have been presented in patients with acrodermatitis chronica atrophicans. Since there could be several years between the tick bite and development of skin lesions, absence of tick bite in patients' history never exclude the diagnosis. Symptoms and different forms of skin involvement in acrodermatitis chronica atrophicans are dependent to duration of the disease. Symptoms, such as sclerotic skin changes, pain and burning, edema and constitutional symptoms have been observed in acrodermatitis chronica atrophicans. Half of patients with acrodermatitis chronica atrophicans experience symptoms of peripheral neuropathy, such as paresthesia and hypesthesia. Symptoms of peripheral neuropathy can occure at the exact site of acrodermatitis chronica atrophicans's lesion or at other sites. Involvement of lower limb is more common compared to the upper limb. In some cases episodic knee joint effusion has been observed.

Physical Examination

Skin examination of acrodermatitis chronica atrophicans's patients include blue, red or brown discoloration, hypopigmentation, indurated plaques and wrinkles, thinning and shining of involved skin. Readily visible veins, edema, ulcers and peeling are usually found. Although the most common location of these skin changes are observed on limbs, there are some cases with facial and abdominal involvement. Peripheral neuropathy develops in 50% of patients. Physical examination of some patients may reveal ulnar bands. Moreover fibrotic nodules could be seen on bony prominences, such as tibia or ulna.

Laboratory Findings

High anti-spirochetal antibody levels (such as IgG, IgM and IgA) has been detected at indirect immunofluorescence and enzyme linked immunosorbent assay (ELISA). Among various antigens in borrelia burgdorferi, flagellum antigen is one of the recommended serologic evaluation in acrodermatitis chronica atrophicans patients. Diagnosis of acrodermatitis chronica atrophicans can be excluded if the serologic evaluataion is negative. Borrelia itself has been found in some of the skin samples. When clinical presentations are not clear enough, biopsy and histological evaluation can assist. Findings such as plasma cells, histiocytes and lymphocytic infiltration plus telangiectasia and thinning of dermis and epidermis are commonly found in skin biopsies.

Other Diagnostic Studies

There are no other diagnostic studies associated with acrodermatitis chronica atrophicans.

Treatment

Medical Therapy

Antibiotic therapy is recommended in patients with acrodermatitis chronica atrophicans. Up to four weeks treatment with antibiotics such as amoxicillin, doxycycline, ceftriaxone, cefotaxime and penicillin G has been recommended for acrodermatitis chronica atrophicans's treatment.

Primary Prevention

Since transmission of borrelia infection occurs by ticks, mosquitos and horse flies bites, primary prevention could be achieved by bite avoidance. Instructions such as using insect repellants, avoiding tick-infested regions or wearing long sleeves and pants when necessary can help.

Secondary Prevention

Proper antibiotic treatment of a patient who has been diagnosed with lyme disease can reduce the probability of acrodermatitis chronica atrophicans.

References

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