Acrodermatitis Chronica Atrophicans Treatment & Management

  • Author: Bozena Chodynicka, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: May 14, 2012
 

Approach Considerations

The choice of treatment for acrodermatitis chronica atrophicans (ACA) depends on the coexistence of other signs or symptoms of Lyme borreliosis. One should also take into account the results of serologic tests. If not treated early, ACA can be associated with joint manifestations and persistent finger deformities.

Appropriate consultations (ie, neurologist, ophthalmologist, rheumatologist, cardiologist) should be sought if extracutaneous signs and symptoms are present. ACA patients without concurrent extracutaneous disease do not require hospitalization.

The US Food and Drug Administration (FDA) approved a vaccine against Lyme borreliosis for distribution in the United States in January 1999, but it is not currently in general use. 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.

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Pharmacologic Therapy

If extracutaneous Lyme borreliosis signs are absent and the level of specific antibodies is low, the authors usually recommend oral doxycycline or oral amoxicillin administered over a period of 3 weeks.

If organic or systemic physical or laboratory signs of Lyme borreliosis are present or if the antibody titer is high, appropriate treatment should be initiated, typically with ceftriaxone or cefotaxime or aqueous penicillin G given intravenously (IV) for 21-28 days.

The possibility of another concurrent infection (eg, babesiosis, ehrlichiosis, or tick-borne encephalitis) must be kept in mind. About 10% of patients with Lyme disease in southern New England are coinfected with babesiosis, and about the same percentage in parts of the Midwestern United States have human granulocytic ehrlichiosis (HGE). Unlike ehrlichiosis, which is usually a short-lived infection, babesiosis can be persistent and may coexist with ACA.

Patients in the early phase of ACA should be assured that resolution of symptoms may occur gradually over a period of several weeks or months after treatment (see the images below); patients treated in the atrophic phase should be informed that although disease progression can be stopped, the symptoms may be only partially reversible.

After 30 days of treatment with ceftriaxone. After 30 days of treatment with ceftriaxone. The same patient after treatment. The same patient after treatment.
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Long-Term Monitoring

Initially, follow-up care should be performed every 3-6 months; later, it may be performed once a year. Physical examination for signs and symptoms of cutaneous and extracutaneous manifestations of Lyme borreliosis is important. In most ACA patients who have received antibiotic therapy, quantitative serologic tests show IgG antibody titers that either do not change or decline only to a certain extent.

Despite the persistence of IgG antibody response in late Lyme borreliosis (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.

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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, University of Medicine and Dentistry of New Jersey-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, MD  Assistant Professor, Department of Dermatology and Venereology,Medical University of Bialystok, Poland

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

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, Northside 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.

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.

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.

References

  1. Bhate C, Schwartz RA. Lyme disease: Part I. Advances and perspectives. J Am Acad Dermatol. Apr 2011;64(4):619-36; quiz 637-8. [Medline].

  2. Bhate C, Schwartz RA. Lyme disease: Part II. Management and prevention. J Am Acad Dermatol. Apr 2011;64(4):639-53; quiz 654, 653. [Medline].

  3. Buchwald A. Ein Fall von diffuser idiopathischer Haut-Atrophie. Vrtljschr Derm. 1883;10:553-6.

  4. Herxheimer K, Hartmann K. Ueber Acrodermatitis chronica atrophicans. Arch f Dermatol u Syph (Wien). 1902;61:57-76.

  5. Smetanick MT, Zellis SL, Ermolovich T. Acrodermatitis chronica atrophicans: a case report and review of the literature. Cutis. May 2010;85(5):247-52. [Medline].

  6. Aberer E. Country report - Austria. Report of WHO Workshop on Lyme Borreliosis Diagnosis and Surveillance, Warsaw. Warsaw, Poland: World Health Organization; 1995.

  7. 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].

  8. Flisiak I, Schwartz RA, Chodynicka B. Clinical features and specific immunological response to Borrelia afzelii in patients with acrodermatitis chronica atrophicans. J Med. 1999;30(3-4):267-78. [Medline].

  9. 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].

  10. Andres C, Ziai M, Bruckbauer H, Ring J, Hofmann H. Acrodermatitis chronica atrophicans in two children. Int J Dermatol. Feb 2010;49(2):180-3. [Medline].

  11. Leverkus M, Finner AM, Pokrywka A, Franke I, Gollnick H. Metastatic squamous cell carcinoma of the ankle in long-standing untreated acrodermatitis chronica atrophicans. Dermatology. 2008;217(3):215-8. [Medline].

  12. Danz B, Kreft B, Radant K, Marsch WCh, Fiedler E. Skin-coloured facial oedema as an initial manifestation of acrodermatitis chronica atrophicans. J Eur Acad Dermatol Venereol. Jun 2008;22(6):751-3. [Medline].

  13. Mullegger RR, Glatz M. Skin manifestations of lyme borreliosis: diagnosis and management. Am J Clin Dermatol. 2008;9(6):355-68. [Medline].

  14. Maraspin V, Ogrinc K, Ružic-Sabljic E, Lotric-Furlan S, Strle F. Isolation of Borrelia burgdorferi sensu lato from blood of adult patients with borrelial lymphocytoma, Lyme neuroborreliosis, Lyme arthritis and acrodermatitis chronica atrophicans. Infection. Feb 2011;39(1):35-40. [Medline].

 
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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.
A 73-year-old female farmer 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.
The external part of the right foot.
A 50-year-old male farmer 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.
The typical inflammatory phase patches are seen on the right hand bone prominences.
A 68-year-old woman 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.
After 30 days of treatment with ceftriaxone.
The livedo racemosa and acrodermatitis chronica atrophicans lesions on the left thigh and buttock before treatment.
The same patient after treatment.
A 69-year-old woman. 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.
The inflammatory phase of acrodermatitis chronica atrophicans can be seen with rosacea lesions on the cheek, the forehead, and the nose.
Fibrotic nodules on the left elbow.
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.
A 26-year-old female nurse 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.
Atrophic phase of acrodermatitis chronica atrophicans of the right upper limb with induration of the forearm.
A 68-year-old female jogger 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.
A widespread acrodermatitis chronica atrophicans atrophic plaque on the back.
The atrophic skin lesions and fibrotic nodules of the right upper limb.
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.
A higher magnification. Telangiectatic vessels are surrounded by a cellular infiltrate in the upper dermis.
A higher magnification of the same biopsy specimen. Note the patchy cell infiltrate around the large vessels in the deep dermis.
 
 
 
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