AUTHOR AND EDITOR INFORMATION

Section 1 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

INTRODUCTION

Section 2 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

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 1883; Herxheimer and Hartmann described it in 1902 as a tissue paper–like cutaneous atrophy. 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 ACA. Another genospecies of the Borrelia burgdorferi sensu lato complex, Borrelia garinii, has also been detected.

ACA is the only form of LB in which no spontaneous remission occurs. Its pathophysiology is not yet fully understood. ACA 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 LB.

A restricted pattern of cytokine expression in ACA, 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 ACA is connected with the ecology of LB, which varies in different geographical regions of the world.

Despite a high incidence of LB 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]), ACA is not seen in the United States, except in a few European immigrants.

International

The occurrence of ACA is connected with the ecology of LB, 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 LB arthritis.

Tick vectors of B afzelii, the main etiologic agent of ACA (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 LB.

In Europe, LB 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).

The frequency of ACA is about 1-10% of all European patients with LB, varying according to the region of the population sampled. Among the group of patients with skin manifestations of LB observed in Vienna, the ratio of the number of EM cases to ACA 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 LB in northeastern Poland).

Because the clinical diagnosis of ACA is much more difficult than that of EM or BL, the condition is often underdiagnosed, and, in fact, the ratio of EM cases to ACA cases may be higher. The total number of cases could increase with increasing frequency of untreated European LB. ACA 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 ACA were rare (0.3%) (Christova, 2004).

Mortality/Morbidity

The course of ACA 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 ACA to be a precancerous disorder.
• The general status of patients with ACA remains good, though they may experience neurologic and/or rheumatologic signs and symptoms.

Race

ACA 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 ACA are women. Among the authors' 19 patients, only 5 were men (Flisiak, 1999).

Age

The disease 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 (Flisiak, 1999).
• 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.
• ACA 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.

CLINICAL

Section 3 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

History

Because of its late onset, patients with ACA 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. ACA 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 LB; 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. ACA is most often unilateral, although bilateral ACA is also common.
• Progressive allodynia, the exaggerated reaction to pain, is a characteristic symptom and, thus, may be a clue to the diagnosis of ACA.
• Patients commonly complain of spontaneous acral pain and paresthesia or dysesthesia or cognitive dysfunction.
• ACA 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.
• • ACA 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 ACA or develop simultaneously. The most common sites of these nodules are the elbows and the knees.
• ACA 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 ACA develop sclerodermalike plaques. Anetodermalike skin lesions can be seen concomitant with ACA.
• ACA 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 ACA 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 ACA 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 (see Image 1).
  • Skin changes are often associated with regional or generalized lymphadenopathy.
• The later, atrophic phase of ACA 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 ACA 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 ACA 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.
• ACA 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 ACA was a 68-year-old woman first seen with prominent livedo racemosa on the leg where typical ACA inflammatory phase patches developed (Flisiak, 1999). Others also observed the same phenomenon, so perhaps this may be more than a chance linkage.
• Detailed clinical and neurophysiologic examinations in patients with ACA-associated polyneuropathy often show a sensory polyneuropathy.
• • Neuropathy symptoms, most often pain and/or paresthesia, are evident in one half of patients with ACA.
  • One of the authors' patients had paresis of the brachial plexus (Flisiak, 1999).
  • 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 ACA lesions.
  • Abnormalities in cerebrospinal fluid seldom have been found in patients with ACA.
• ACA 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 LB facilitates ACA development.

DIFFERENTIALS

Section 4 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

WORKUP

Section 5 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Lab Studies

• Genetic profiles of strains of B burgdorferi sensu lato derived from patients with 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.
• Physicians should diagnose ACA based on careful evaluation of the history, including epidemiologic data, signs, and symptoms of early and late infection; on a detailed physical examination; and on the specific serologic tests results and 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 polymerase chain reaction (PCR), but both cultures and direct spirochetal stains are usually negative.
• Laboratory evidence of infection by demonstration of specific antibodies with a 2-test approach involving initial screening with enzyme-linked immunosorbent assay (ELISA) or indirect immunofluorescence assay (IFA) and subsequent confirmation of positive and equivocal results with Western blot is essential for diagnosis of LB. All patients with ACA are seropositive for immunoglobulin G (IgG), some of them also for immunoglobulin M (IgM) antiborrelial antibodies.
• False-positive results of IFA or ELISA can occur because of cross-reactivity with Treponema pallidum, saprophytic Treponema and other spirochetal agents (ie, Leptospira species), and rheumatoid factor. False-positive results may also occur in patients with infectious mononucleosis and other disorders with activated B cells.
• • In the regions in which a high incidence of syphilis exists (ie, Eastern Europe, Central Asia), in every case of B burgdorferi seropositivity, T pallidum infection should be excluded using the Treponema pallidum hemagglutination assay (TPHA). Infection of B burgdorferi should also be ruled out in cases of false-positive serologic tests results for syphilis.
  • ELISA with sonicated or purified or recombinant antigens of B burgdorferi seems to be more specific than IFA using cultured borreliae and serum preexposed to nonpathogenic Treponema phagedenis.
• Immunoblotting with various B burgdorferi antigens is used as a confirmatory test in LB.
• • The authors found that the immunoblot technique using B afzelii flagellar antigen (41 kd) is useful in the diagnosis of ACA (Flisiak, 1999).
  • In the authors' series of 9 patients, all were positive with IgG and 5 of them were also positive with IgM (Flisiak, 1999).
• In the authors' opinion, the strategy of the serologic diagnosis of ACA (as in the cases of late syphilis) should include the assessment of quantitative serologic test results (ie, IFA) because a high titer of antibodies, even in persons without clinical signs and symptoms of neuroborreliosis, may point to CNS involvement and to the need for cerebrospinal fluid examination for intrathecal production of antiborrelial antibodies.

Imaging Studies

• Radiologic findings in patients with ACA may show subluxation of the toe joint and periostitis of the bones of the lower limb.

Other Tests

• Sural nerve biopsy may demonstrate a mainly axonal neuropathy.
• Cerebrospinal fluid testing shows mononuclear pleocytosis and intrathecal production of specific antibodies when the CNS is involved.

Histologic Findings

The most important histologic findings in ACA are the presence of telangiectasias and cellular infiltrates of lymphocytes with admixed plasma cells in the absence of any other explanation for the plasma cells (eg, syphilis, myeloma). Though not diagnostic, these changes are highly suggestive.

ACA in its early inflammatory edematous stage shows a dense, patchy perivascular and periappendiceal dermal infiltrate of lymphocytes, histiocytes, and plasma cells. Collagen bundles become swollen and homogeneous and are split by mucinous deposition.

The atrophic stage of 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 is often not the case.

Immunohistochemical studies show few B cells despite a substantial number of plasma cells.

TREATMENT

Section 6 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Medical Care

The choice of treatment depends on the coexistence of other signs or symptoms of LB with ACA. The authors also consider the value of the titer of serologic tests.

• If the extracutaneous LB signs are absent and the level of specific antibodies is low, the authors usually recommend oral doxycycline or oral amoxicillin, over 3 weeks.
• If organic or systemic physical or laboratory signs of LB are present or if the antibody titer is high, the appropriate treatment should be introduced mainly with ceftriaxone or cefotaxime or aqueous penicillin G given intravenously for 21-28 days.

Consultations

Seek the appropriate consultations (ie, neurologist, ophthalmologist, rheumatologist, cardiologist) if extracutaneous signs and symptoms exist.

MEDICATION

Section 7 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

The goals of pharmacotherapy are to eradicate the infection, to reduce morbidity, and to prevent complications.

Drug Category: Antibiotics

Empiric antimicrobial therapy must be comprehensive and should cover all likely pathogens in the context of this clinical setting.

FOLLOW-UP

Section 8 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Further Inpatient Care

• Patients with ACA without concurrent extracutaneous disease do not require hospitalization.
• Consider a possible concurrent infection.
• • Co-infections include babesiosis, ehrlichiosis, and tick-borne encephalitis.
  • About 10% of patients with Lyme disease in southern New England are co-infected with babesiosis, and about the same percentage in parts of the midwestern United States have human granulocytic ehrlichiosis.
  • Babesiosis can cause a persistent infection and coexist with ACA, unlike ehrlichiosis, which is usually a short-lived infection.

Further Outpatient Care

• Assure patients in the early phase of ACA that the resolution of symptoms may occur gradually during several weeks or months after treatment.
• Inform patients treated in the atrophic phase that the disease progression can be stopped, but the symptoms may be only partially reversible.
• Follow-up care should be performed initially every 3-6 months and later once a year.
• • Physical examination for signs and symptoms of cutaneous and extracutaneous manifestations of LB is important.
  • Quantitative serologic tests show a lack of changes in IgG antibody titer or show it declining only to a certain extent in most patients with ACA after antibiotic therapy.
• Despite the persistence of IgG antibody response in late LB (serologic scar) similar to that observed in syphilis cases, the authors do serologic follow-up testing according to the recommendation for late syphilis. A sudden increase in antibody titer can point to the activation of infection and precede a clinical recurrence of the disease.

Deterrence/Prevention

• The Food and Drug Administration (FDA) approved a vaccine against LB for distribution in the United States in January 1999.
• At present, for European countries, a recombined vaccine is being prepared from the surface lipoprotein A (OspA) made from prevalent strains of B afzelii and B garinii.

Complications

• Sequelae are rarely observed in patients with ACA but include bacterial superinfections and chronic ulcerations of atrophic skin and fibrotic changes in the joints.

Prognosis

• The outlook is good if the acute inflammatory stage of ACA is treated adequately.
• The therapeutic outcome is difficult to assess in patients with the chronic atrophic phase, in which many changes are only partially reversible.

Patient Education

• Educate patients and their families about how to lower the risk of acquiring this disorder.
• • Persons residing in endemic areas should examine their bodies for ticks every 12 hours and expeditiously remove any attached ticks.
  • Persons should use personal protection (eg, light-colored clothing to facilitate tick visualization) and tuck pant cuffs into socks to discourage ticks from accessing exposed skin.
• For excellent patient education resources, visit eMedicine's Bacterial and Viral Infections Center and Bites and Stings Center. Also, see eMedicine's patient education articles Lyme disease and Ticks.

MISCELLANEOUS

Section 9 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Medical/Legal Pitfalls

• If not treated early, ACA can be associated with joint manifestations and persistent finger deformities.

MULTIMEDIA

Section 10 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Media file 1:  The most common localization of the skin lesions in 12 patients with acrodermatitis chronica atrophicans (ACA). The number of ACA lesions in the particular body region is shown.
	View Full Size Image
Media type:  Image

Media file 2:  A 73-year-old female farmer (same patient as in Image 3) with a cutaneous plaque on the sole of her right foot lasting for 6 months that, in the meantime, had extended onto the dorsum of her foot, her right leg, and the lower part of her right thigh. Infection of Borrelia burgdorferi was diagnosed with enzyme-linked immunosorbent assay, and acrodermatitis chronica atrophicans was confirmed histologically.
	View Full Size Image
Media type:  Photo

Media file 3:  The external part of the right foot on the same patient as in Image 2.
	View Full Size Image
Media type:  Photo

Media file 4:  A 50-year-old male farmer (same patient as in Image 5) was examined for cutaneous plaques on the dorsal side of his right hand lasting for 8 months that, in the meantime, had extended onto his right forearm and arm and had also developed on his right thigh. The patient complained of muscular weakness related to his right upper limb and periodic arthralgia. The neurologic examination demonstrated signs of right brachial plexus damage, confirmed by electromyography. Borrelia burgdorferi infection was diagnosed with enzyme-linked immunosorbent assay, indirect immunofluorescence assay (titer: 1:1,024), and Western blot. Histologic examination confirmed the diagnosis of acrodermatitis chronica atrophicans.
	View Full Size Image
Media type:  Photo

Media file 5:  The typical inflammatory phase patches are seen on the right hand bone prominences of the same patient as in Image 4.
	View Full Size Image
Media type:  Photo

Media file 6:  A 68-year-old woman (same patient as in Images 7-9) with a history of untreated erythema migrans on her left thigh 2 years previously. Ten months later, the plaque extended over the skin of her left buttock and became bluish with signs of livedo racemosa. Her forearms and breast were also involved. Borrelia burgdorferi infection was diagnosed with indirect immunofluorescence assay (1:4,096) and Western blot. Acrodermatitis chronica atrophicans was confirmed histologically. Because of intrathecal production of specific antibodies, diagnosis of asymptomatic neuroborreliosis was established.
	View Full Size Image
Media type:  Photo

Media file 7:  The same patient as in Images 6 and 8-9 after 30 days of treatment with ceftriaxone.
	View Full Size Image
Media type:  Photo

Media file 8:  The livedo racemosa and acrodermatitis chronica atrophicans lesions on the left thigh and buttock of the same patient as in Images 6-7 and 9 before treatment.
	View Full Size Image
Media type:  Photo

Media file 9:  The same patient as in Images 6-8 after treatment.
	View Full Size Image
Media type:  Photo

Media file 10:  A 69-year-old woman (same patient as in Images 11-12). The initial lesion developed on the dorsal side of her left hand 2 years previously and extended onto her left forearm and arm. A new erythematous lesion developed 2 months before on her right cheek beside the rosacea signs. Acrodermatitis chronica atrophicans was confirmed in the biopsy specimen taken from the skin of the forearm, and Borrelia burgdorferi infection was diagnosed with indirect immunofluorescence assay (1:2,048). The atrophic phase of acrodermatitis chronica atrophicans is visible on the hand, and the inflammatory phase is visible on the cheek.
	View Full Size Image
Media type:  Photo

Media file 11:  The inflammatory phase of acrodermatitis chronica atrophicans can be seen with rosacea lesions on the cheek, the forehead, and the nose of the same patient as in Images 10 and 12.
	View Full Size Image
Media type:  Photo

Media file 12:  Fibrotic nodules on the left elbow of the same patient as in Images 10-11.
	View Full Size Image
Media type:  Photo

Media file 13:  A 48-year-old woman with a history of frequent tick bites and an initial inflammatory skin lesion on the left medial part of her ankle 2 years previously. The lesion extended onto the left leg and involved the knee. Fibrotic nodules developed in the medial part of the ankle and the knee. Moreover, she complained of balance disturbances and vertigo. Neurologic examination revealed the asymmetry of profound reflexes, bilateral lack of plantar reflexes with a tendency to the extensor plantar response (Babinski sign), ataxia, profound dysesthesia, and muscular atrophy of the left calf. Acrodermatitis chronica atrophicans was confirmed by histologic examination, and Borrelia burgdorferi infection was confirmed by a high specific antibody titer with indirect immunofluorescence assay (1:8,192); cerebrospinal fluid was not tested.
	View Full Size Image
Media type:  Photo

Media file 14:  A 26-year-old female nurse (same patient as in Image 15) recalled the onset of the disease on her right arm 4 years before. After 6 months, the plaques extended onto the right forearm and hand. The left arm and forearm were also involved 3 years previously. Induration of the skin of the right forearm was noted. Borrelia burgdorferi infection was diagnosed with indirect immunofluorescence assay (1:2,048) and confirmed by positive Western blot for both immunoglobulin M and immunoglobulin G antibodies.
	View Full Size Image
Media type:  Photo

Media file 15:  Atrophic phase of acrodermatitis chronica atrophicans of the right upper limb with induration of the forearm of the same patient as in Image 14.
	View Full Size Image
Media type:  Photo

Media file 16:  A 68-year-old female jogger (same patient as in Images 17-18) frequently exposed to ticks. Cutaneous plaques developed 4 years previously on her right lower limb and the right part of her trunk, including her breast and right upper limb. Typical extensive cigarette paper–like plaques, bluish or brownish red in color were evident. Fibrous nodules were found on her right elbow. Borrelia burgdorferi infection was confirmed with indirect immunofluorescence assay (1:1,024) and Western blot. Acrodermatitis chronica atrophicans was finally diagnosed by using histologic examinations.
	View Full Size Image
Media type:  Photo

Media file 17:  A widespread acrodermatitis chronica atrophicans atrophic plaque on the back of the same patient as in Images 16 and 18.
	View Full Size Image
Media type:  Photo

Media file 18:  The atrophic skin lesions and fibrotic nodules of the right upper limb of the same patient as in Images 16-17.
	View Full Size Image
Media type:  Photo

Media file 19:  Biopsy specimen from the wrist of the patient shown in Image 4. The epidermis is slightly flattened. A zone of normal connective tissue can be seen below the epidermis. A patchy infiltrate consisting of lymphocytes and plasma cells is seen throughout the dermis. Telangiectasias are evident in the upper and deeper parts of the dermis.
	View Full Size Image
Media type:  Photo

Media file 20:  A higher magnification of the same biopsy specimen as in Images 19 and 21. Telangiectatic vessels are surrounded by a cellular infiltrate in the upper dermis.
	View Full Size Image
Media type:  Photo

Media file 21:  A higher magnification of the same biopsy specimen as in Images 19-20. Note the patchy cell infiltrate around the large vessels in the deep dermis.
	View Full Size Image
Media type:  Photo

REFERENCES

Section 11 of 11

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

• Aberer E, Breier F, Stanek G, Schmidt B. Success and failure in the treatment of acrodermatitis chronica atrophicans. Infection. Jan-Feb 1996;24(1):85-7. [Medline].
• Aberer E, Kersten A, Klade H, et al. Heterogeneity of Borrelia burgdorferi in the skin. Am J Dermatopathol. Dec 1996;18(6):571-9. [Medline].
• Aberer E. Country report - Austria. Report of WHO Workshop on Lyme Borreliosis Diagnosis and Surveillance, Warsaw,. WHO/CDS/VPH/95;33-40.
• Asbrink E. Cutaneous manifestations of Lyme borreliosis. Clinical definitions anddifferential diagnoses. Scand J Infect Dis Suppl. 1991;77:44-50. [Medline].
• Asbrink E, Hovmark A. Comments on the course and classification of Lyme borreliosis. Scand J Infect Dis Suppl. 1991;77:41-3. [Medline].
• Baumann M, Tebbe B, Arnold M, et al. [Livedo racemosa: an unusual late manifestation of borreliosis?]. Hautarzt. Aug 2000;51(8):593-6. [Medline].
• Braun-Falco O, Plewig G, Wolff HH. Dermatology. 2nd ed. Berlin: Springer-Verlag;2000:188-190.
• Brehmer-Andersson E, Hovmark A, Asbrink E. Acrodermatitis chronica atrophicans: histopathologic findings and clinical correlations in 111 cases. Acta Derm Venereol. 78(3):207-13. [Medline].
• Brzonova I, Wollenberg A, Prinz JC. Acrodermatitis chronica atrophicans affecting all four limbs in an 11-year-old girl. Br J Dermatol. Aug 2002;147(2):375-8. [Medline].
• Buchwald A. Ein Fall von diffuser idiopathischer Haut-Atrophie. Vrtljschr Derm. 1883;10:553-556.
• Chodynicka B, Flisiak I. Treatment and prophylaxis of Lyme disease. Postepy Dermatol (Poznan). 2000;17:43-52.
• Chodynicka B, Lukaszuk C, Pucilo K. Studies on the value of IFA and ELISA in the screening and diagnosing of Lyme borreliosis. Przegl Dermatol. 1996;83:159-161.
• Chodynicka B, Flisiak I, Lukaszuk C, Bulhak V. [Late consequences of untreated lyme borreliosis]. Przegl Epidemiol. 1997;51(4):445-9. [Medline].
• Christova I, Komitova R. Clinical and epidemiological features of Lyme borreliosis in Bulgaria. Wien Klin Wochenschr. Jan 31 2004;116(1-2):42-6. [Medline].
• Dh te R, Basse-Guerineau AL, Beaumesnil V, et al. Full spectrum of clinical, serological, and epidemiological features ofcomplicated forms of Lyme borreliosis in the Paris, France, area. Eur J Clin Microbiol Infect Dis. Nov 2000;19(11):809-15. [Medline].
• DiCaudo DJ, Su WP, Marshall WF, et al. Acrodermatitis chronica atrophicans in the United States: clinical and histopathologic features of six cases. Cutis. Aug 1994;54(2):81-4. [Medline].
• Fingerle V, Wilske B. Stage-oriented treatment of Lyme borreliosis. MMW Fortschr Med. 2006;148:39-41. [Medline].
• Flisiak I, Schwartz RA, Chodynicka B. Clinical features and specific immunological response against Borrelia afzelii in patients with acrodermatitis chronica atrophicans. J Med. 1999;30:267-278.
• Gerster JC, Peter O. Rheumatic manifestations related to acrodermatitis chronica atrophicans. A review of four cases. Rev Rhum Engl Ed. Oct 1998;65(10):567-70. [Medline].
• Herxheimer K, Hartmann K. Ueber Acrodermatitis chronica atrophicans. Arch f Dermatol u Syph (Wien). 1902;61:57-76.
• Hodl S, Soyer HP. Dermatopathology of Lyme disease. Acta Derm Venerol (Ljubljana). 1994;3:89-98.
• Houtman PM, Tazelaar DJ. Joint and bone involvement in Dutch patients with Lyme borreliosis presenting with acrodermatitis chronica atrophicans. Neth J Med. Jan 1999;54(1):5-9. [Medline].
• Kansky A. Lyme disease is spreading to the South-East. Skin manifestations in Croatia 1988-1990. Acta Derm Venerol (Ljubljana). 1992;1:93-95.
• Kindstrand E, Nilsson BY, Hovmark A, et al. Polyneuropathy in late Lyme borreliosis - a clinical, neurophysiological and morphological description. Acta Neurol Scand. 101(1):47-52. [Medline].
• Kindstrand E, Nilsson BY, Hovmark A, et al. Peripheral neuropathy in acrodermatitis chronica atrophicans - a late Borrelia manifestation. Acta Neurol Scand. 95(6):338-45. [Medline].
• Maraspin V, Ruzic-Sabljic E, Strle F. Isolation of Borrelia burgdorferi sensu lato from a fibrous nodule in apatient with acrodermatitis chronica atrophicans. Wien Klin Wochenschr. Jul 31 2002;114(13-14):533-4. [Medline].
• Mullegger RR, McHugh G, Ruthazer R, et al. Differential expression of cytokine mRNA in skin specimens from patientswith erythema migrans or acrodermatitis chronica atrophicans. J Invest Dermatol. Dec 2000;115(6):1115-23. [Medline].
• Oschmann P. Lyme borreliosis and tick-borne encephalitis. In: Oschmann P, Kraiczy P, Halperin J, Brade V, eds. UNI-MED Verlag AG, Bremen. 1999.
• Philipson A. Antibiotic treatment in Lyme borreliosis. Scand J Infect Dis Suppl. 1991;77:145-50. [Medline].
• Picken RN, Strle F, Picken MM, et al. Identification of three species of Borrelia burgdorferi sensu lato (B. burgdorferi sensu stricto, B. garinii, and B. afzelii) among isolates from acrodermatitis chronica atrophicans lesions. J Invest Dermatol. Mar 1998;110(3):211-4. [Medline].
• Ruzic-Sabljic E, Podreka T, Maraspin V, Strle F. Susceptibility of Borrelia afzelii strains to antimicrobial agents. Int J Antimicrob Agents. 2005;25:474-8. [Medline].
• Schmidt BL, Aberer E, Stockenhuber C, et al. Detection of Borrelia burgdorferi DNA by polymerase chain reaction in theurine and breast milk of patients with Lyme borreliosis. Diagn Microbiol Infect Dis. Mar 1995;21(3):121-8. [Medline].
• Zalaudek I, Leinweber B, Kerl H, Mullegger RR. Acrodermatitis chronica atrophicans in a 15-year-old girl misdiagnosed as venous insufficiency for 6 years. J Am Acad Dermatol. 2005;52:1091-4. [Medline].
• de Koning J, Tazelaar DJ, Hoogkamp-Korstanje JA, Elema JD. Acrodermatitis chronica atrophicans: a light and electron microscopic study. J Cutan Pathol. Feb 1995;22(1):23-32. [Medline].

Article Last Updated: Oct 6, 2006