eMedicine Specialties > Dermatology > Bacterial Infections

Acrodermatitis Chronica Atrophicans

Author: Bozena Chodynicka, MD, Head, Professor, Department of Dermatology and Venereology, Medical University of Bialystok, Poland
Coauthor(s): Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School; Iwona Flisiak, assistant, MD, Department of Dermatology and Venereology, Medical University of Bialystok, Poland
Contributor Information and Disclosures

Updated: Mar 23, 2009

Introduction

Background

Acrodermatitis chronica atrophicans (ACA) is the third or late stage of European Lyme borreliosis (LB). This unusual, progressive, fibrosing skin process is due to the effect of continuing active infection with Borrelia afzelii. Buchwald first delineated it in 1883¹ ; Herxheimer and Hartmann described it in 1902 as a tissue paper–like cutaneous atrophy.² It is evident on the extremities, particularly on the extensor surfaces, beginning with an inflammatory stage with bluish red discoloration and cutaneous swelling and concluding 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.

Pathophysiology

B afzelii is the predominant, but may not be the exclusive, etiologic agent of acrodermatitis chronica atrophicans. Another genospecies of the Borrelia burgdorferi sensu lato complex, Borrelia garinii, has also been detected.

Acrodermatitis chronica atrophicans is the only form of Lyme borreliosis in which no spontaneous remission occurs. Its pathophysiology is not yet fully understood. Acrodermatitis chronica atrophicans appears to be associated with long-term persistence of Borrelia organisms in the skin; several nonspecific reactions together with a specific immune response may contribute to its manifestations.

The persistence of the spirochetes despite a marked cutaneous T-cell infiltration and high serum antibody titers may be connected with resistance of the pathogen to the complement system; the ability to escape to immunologically protected sites (eg, endothelial cells, fibroblasts); and the ability to change antigens, which may lead to an inappropriate immune response. Lack of protective antibodies, with a narrow antibody spectrum and a weak cellular response with down-regulation of major histocompatibility system class II molecules on Langerhans cells, has been observed in patients with Lyme borreliosis.

A restricted pattern of cytokine expression in acrodermatitis chronica atrophicans, including the lack of interferon-gamma, may contribute to its chronicity. Cross-reactive antibody responses could take part in autoimmune damage, but whether autoimmune reactions play any role in the pathogenesis of the disease is unclear. The pathogenic mechanism of atrophic skin changes has also not been clarified. Perhaps periarticular regions are favorite sites because of reduced acral skin temperatures or reduced oxygen pressure.

Frequency

United States

The occurrence of acrodermatitis chronica atrophicans is connected with the ecology of Lyme borreliosis, which varies in different geographical regions of the world.

Despite a high incidence of Lyme borreliosis in the United States (varying from 95 cases per 100,000 population in Connecticut to 1250 cases per 100,000 population in Nantucket County, Massachusetts [1996 data]), acrodermatitis chronica atrophicans is not seen in the United States, except in a few European immigrants.

International

The occurrence of acrodermatitis chronica atrophicans is connected with the ecology of Lyme borreliosis, which varies in different geographical regions of the world.

Ixodes scapularis, Ixodes pacificus, and 4 other tick species distributed in North America transmit B burgdorferi sensu stricto, causing EM and Lyme borreliosis arthritis.

Tick vectors of B afzelii, the main etiologic agent of acrodermatitis chronica atrophicans (and erythema migrans [EM]), are Ixodes ricinus, Ixodes hexagonus, and Ixodes persulcatus distributed in western and central Europe and in far eastern Europe and Asia. Almost all of these hard tick species may also transmit B garinii, a causative agent of EM and neurologic symptoms of Lyme borreliosis.

In Europe, Lyme borreliosis with all its dermatologic manifestations occurs in almost all countries, predominantly in the central part of the continent. The annual incidence per 100,000 population varies from 16 cases in France to 120 cases in northeastern Poland and Slovenia and to 130 cases in Austria (1995 data).³

The frequency of acrodermatitis chronica atrophicans is about 1-10% of all European patients with Lyme borreliosis, varying according to the region of the population sampled. Among the group of patients with skin manifestations of Lyme borreliosis observed in Vienna, the ratio of the number of EM cases to acrodermatitis chronica atrophicans cases and to Borrelia lymphocytoma (BL) cases was 30:3:1. This ratio is 170:5:1 in the authors' as-yet-unpublished studies (provided in the group of patients with Lyme borreliosis in northeastern Poland).

Because the clinical diagnosis of acrodermatitis chronica atrophicans is much more difficult than that of EM or BL, the condition is often underdiagnosed, and, in fact, the ratio of EM cases to acrodermatitis chronica atrophicans cases may be higher. The total number of cases could increase with increasing frequency of untreated European Lyme borreliosis. Acrodermatitis chronica atrophicans is probably the most common late and chronic manifestation of the borreliosis in European patients with Lyme disease.

A Bulgarian survey found that borrelial lymphocytoma and acrodermatitis chronica atrophicans were rare (0.3%).⁴

Mortality/Morbidity

The course of acrodermatitis chronica atrophicans is long-standing, lasting from a few to several years, and it leads to extensive flaccid atrophy of the skin and, in some patients, to the limitation of upper and lower limb joint mobility.

• Chronic, difficult-to-treat ulcerations of atrophic skin may develop after minor trauma. Malignant degeneration has rarely been observed; one should not consider acrodermatitis chronica atrophicans to be a precancerous disorder.
• The general status of patients with acrodermatitis chronica atrophicans remains good, though they may experience neurologic and/or rheumatologic signs and symptoms.

Race

Acrodermatitis chronica atrophicans is not limited to any one nationality or race. It is much more frequent in whites than in other races, probably because of a far higher exposure to ticks transmitting B afzelii.

Sex

More than two thirds of patients with acrodermatitis chronica atrophicans are women. Among the authors' 19 patients, only 5 were men.⁵

Age

Acrodermatitis chronica atrophicans  can occur in any age group, but it is most frequent in adults, usually in their 40s or 50s.

• The youngest of the authors' patients was 26 years; the oldest was 73 years.⁵
• The mean age of the female group was 54.3 ± 12.8 years; the mean age of the male group was 46.2 ± 6.5 years.
• Acrodermatitis chronica atrophicans is rare in adolescents; however, it has been observed in children. A case in a 15-year-old girl was reported by Zalaudek et al in 2005.⁶

Clinical

History

Because of its late onset, patients with acrodermatitis chronica atrophicans rarely remember a tick bite. Instead, they recall having been in the woods or grassy areas a few months or years previously, especially in a geographically endemic region. A history of EM is recalled by about 20% of patients. Acrodermatitis chronica atrophicans can develop directly from EM or after 6-36 months, often involving the same region of the body. Sometimes, the disease may be preceded by a latent phase (lasting up to several years) or by other manifestations of Lyme borreliosis; the latter can also develop simultaneously.

• The patient notices localized cutaneous swelling on the distal extremity or on only one of the digits and sometimes discovers that one foot is larger than the other when buying shoes. Acrodermatitis chronica atrophicans is most often unilateral, although bilateral acrodermatitis chronica atrophicans is also common.
• Progressive allodynia, the exaggerated reaction to pain, is a characteristic symptom and, thus, may be a clue to the diagnosis of acrodermatitis chronica atrophicans.
• Patients commonly complain of spontaneous acral pain and paresthesia or dysesthesia or cognitive dysfunction.
• Acrodermatitis chronica atrophicans starts with an inflammatory phase, characterized by few to several soft, erythematous, slowly enlarging cutaneous swellings or flat infiltrations of various sizes or with diffuse bluish red discoloration and edema of the skin.
  • Acrodermatitis chronica atrophicans usually appears on the distal part of at least one extremity, predominantly on the extensor surfaces on the bony prominences.
  • Common sites are the foot, the lower leg or the hand, the forearm, and the olecranon area; however, they uncommonly appear proximally on the upper arm and the shoulder or the thigh and the buttock.
  • Sometimes, the erythema is slight and swelling may dominate, or the signs are very subtle and may be overlooked by the patient or the physician.
  • Lymphadenopathy may be noticed.
• Only one part of an extremity may be affected for many months or years. With time, the skin lesions may extend on one extremity or appear on additional ones and also involve other parts of the body.
• Fibrotic nodules (often multiple, localized linearly in the vicinity of joints) are typical. They can precede acrodermatitis chronica atrophicans or develop simultaneously. The most common sites of these nodules are the elbows and the knees.
• Acrodermatitis chronica atrophicans does not heal spontaneously; gradual conversion into its atrophic phase may occur during many years of infection.
  • The skin becomes thin, atrophic, wrinkled, dry, and translucent.
  • The hair is lost; the number of sebaceous and sweat glands are decreased.
  • Even minor trauma may produce large, slow-to-heal ulcerations of the affected skin.
• About 5-10% of patients with acrodermatitis chronica atrophicans develop sclerodermalike plaques. Anetodermalike skin lesions can be seen concomitant with acrodermatitis chronica atrophicans.
• Acrodermatitis chronica atrophicans is accompanied often by peripheral neuropathy, musculoskeletal pains, and joint damage underneath the cutaneous plaques. Involvement of the small joints of the hands and the feet by the fibrotic reaction is often seen.

Physical

The clinical recognition of acrodermatitis chronica atrophicans may be difficult, even in typical cases. A detailed history, including epidemiologic data, is helpful. Physicians should confirm the clinical diagnosis by histopathologic examination and serologic test results.

• The early, inflammatory phase of acrodermatitis chronica atrophicans is marked by soft, painless, poorly demarcated, bluish reddish plaques tending to coalescence or by diffuse erythema and edema localized on the distal extremities that spread proximally.
  • In the authors' experience, not only the distal extremities but also the proximal parts, the trunk, and the face may be involved in the early stage. Others have also observed skin-colored facial edema as an initial manifestation of acrodermatitis chronica atrophicans.⁷
  • Skin changes are often associated with regional or generalized lymphadenopathy.
• The later, atrophic phase of acrodermatitis chronica atrophicans is more characteristic clinically. The affected skin has a dark red or brownish red discoloration; focal hyperpigmentation; telangiectasias; and a thin, wrinkled, cigarette paper–like, translucent appearance.
  • Because of the loss of subcutaneous fat, the skin vessels become prominent.
  • Atrophy of the epidermis and lack of hairs, sebaceous glands, and often sweat glands make the skin poorly protected and vulnerable.
  • Large ulcerations can be observed, and malignant lesions may also occur.
  • The atrophic poikilodermic changes are often bilateral and most noticeable over the knees, the elbows, and the dorsal surfaces of the hands and the feet. They may also involve the trunk (particularly the chest) and the face.
• Sclerodermalike changes may appear in patients with acrodermatitis chronica atrophicans in both the inflammatory phase and the atrophic phase.
  • These changes are usually limited to the legs and the feet, but they occasionally occur on the trunk.
  • The lesions, similar to morphea and lichen sclerosus and atrophicus, may appear in regions where no acrodermatitis chronica atrophicans is present.
• Single or multiple fibrotic nodules or bands may be seen on the extensor surfaces of the elbows and the knees or adjacent to other joints. They are generally firm; bluish-red, yellowish, or skin-colored; and 0.5 to 2-3 cm in diameter.
• Acrodermatitis chronica atrophicans has been described in association with localized amyloidosis, eczema, psoriasis, lupus erythematosus, leprosy, and Hodgkin disease. These associations may be coincidental. One of the authors' patients with histologically and serologically confirmed acrodermatitis chronica atrophicans was a 68-year-old woman first seen with prominent livedo racemosa on the leg where typical acrodermatitis chronica atrophicans inflammatory phase patches developed.⁵ Others also observed the same phenomenon, so perhaps this may be more than a chance linkage.
• Detailed clinical and neurophysiologic examinations in patients with acrodermatitis chronica atrophicans–associated polyneuropathy often show a sensory polyneuropathy.
  • Neuropathy symptoms, most often pain and/or paresthesia, are evident in one half of patients with acrodermatitis chronica atrophicans.
  • One of the authors' patients had paresis of the brachial plexus.⁵
  • Marked abnormality of the vibratory threshold is a common finding.
  • Patients with localized or asymmetric neuropathy seem to have changes more often found in the extremities with, rather than without, visible acrodermatitis chronica atrophicans lesions.
  • Abnormalities in cerebrospinal fluid seldom have been found in patients with acrodermatitis chronica atrophicans.
• Acrodermatitis chronica atrophicans can produce deformities of the fingers and the toes if it is not treated promptly. Persistent reducible deformities of the fingers may be consistent with Jaccoud arthropathy.

Causes

Lack of adequate or appropriate treatment of early Lyme borreliosis facilitates acrodermatitis chronica atrophicans development.

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