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Acrodermatitis Chronica Atrophicans: Differential Diagnoses & Workup

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

Differential Diagnoses

Endemic Syphilis
Pernio
Eosinophilic Fasciitis
Syphilis
Erysipelas
Systemic Sclerosis
Erysipeloid
Venous Insufficiency
Lichen Sclerosus et Atrophicus
Morphea

Workup

Laboratory Studies

  • Genetic profiles of strains of B burgdorferi sensu lato derived from patients with acrodermatitis chronica atrophicans 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 acrodermatitis chronica atrophicans 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 Lyme borreliosis. All patients with acrodermatitis chronica atrophicans 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 Lyme borreliosis.
    • The authors found that the immunoblot technique using B afzelii flagellar antigen (41 kd) is useful in the diagnosis of acrodermatitis chronica atrophicans.5
    • In the authors' series of 9 patients, all were positive with IgG and 5 of them were also positive with IgM.5
  • In the authors' opinion, the strategy of the serologic diagnosis of acrodermatitis chronica atrophicans (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.
  • Because acrodermatitis chronica atrophicans is often confused with vascular conditions, serologic proof of the diagnosis and histologic verification are considered obligatory8 PCR for Borrelia -specific DNA (rather than culture of the spirochete) is sometimes a necessary adjunct for the diagnosis.

Imaging Studies

  • Radiologic findings in patients with acrodermatitis chronica atrophicans 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 acrodermatitis chronica atrophicans 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.

Acrodermatitis chronica atrophicans 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 acrodermatitis chronica atrophicans 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 acrodermatitis chronica atrophicans. 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.

More on Acrodermatitis Chronica Atrophicans

Overview: Acrodermatitis Chronica Atrophicans
Differential Diagnoses & Workup: Acrodermatitis Chronica Atrophicans
Treatment & Medication: Acrodermatitis Chronica Atrophicans
Follow-up: Acrodermatitis Chronica Atrophicans
Multimedia: Acrodermatitis Chronica Atrophicans
References
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References

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Further Reading

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Keywords

acrodermatitis chronica atrophicans, Herxheimer disease, ACA, European Lyme borreliosis, LB, Lyme disease, borreliosis, Borrelia afzelii, B afzelii, Borrelia garinii, B garinii, Borrelia burgdorferi, B burgdorferi, Ixodes ricinus, I ricinus, Ixodes hexagonus, I hexagonus, Ixodes persulcatus, I persulcatus, Ixodes scapularis, I scapularis, Ixodes pacificus, I pacificus, cutaneous atrophy, erythema migrans, EM, tick bite, tick vector

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Contributor Information and Disclosures

Author

Bozena Chodynicka, MD, Head, Professor, Department of Dermatology and Venereology, Medical University of Bialystok, Poland
Bozena Chodynicka, MD is a member of the following medical societies: Central Neuropsychiatric Association
Disclosure: Nothing to disclose.

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
Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi
Disclosure: Nothing to disclose.

Iwona Flisiak, assistant, MD, Department of Dermatology and Venereology, Medical University of Bialystok, Poland
Disclosure: Nothing to disclose.

Medical Editor

Peter Fritsch, MD, Chair, Department of Dermatology and Venereology, University of Innsbruck, Austria
Peter Fritsch, MD is a member of the following medical societies: American Dermatological Association, International Society of Pediatric Dermatology, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

Pharmacy Editor

David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic
David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Managing Editor

Lester F Libow, MD, Dermatopathologist, South Texas Dermatopathology Laboratory
Lester F Libow, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Texas Medical Association
Disclosure: Nothing to disclose.

CME Editor

Glen H Crawford, MD, Assistant Clinical Professor, Department of Dermatology, University of Pennsylvania School of Medicine; Chief, Division of Dermatology, The Pennsylvania Hospital
Glen H Crawford, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Phi Beta Kappa, and Society of USAF Flight Surgeons
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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