Acrodermatitis chronica atrophicans

(Redirected from Primary diffuse atrophy)

Acrodermatitis chronica atrophicans (ACA) is a skin rash indicative of the third or late stage of European Lyme borreliosis.

Acrodermatitis chronica atrophicans
Other namesHerxheimer disease[1]: 1102  and Primary diffuse atrophy[2]: 293 
SpecialtyDermatology Edit this on Wikidata
Causesuntreated infection with Borrelia afzelii

ACA is a dermatological condition that takes a chronically progressive course and finally leads to a widespread atrophy of the skin. Involvement of the peripheral nervous system is often observed, specifically polyneuropathy.

This progressive skin process is due to the effect of continuing active infection with the spirochete Borrelia afzelii, which is the predominant pathophysiology.[3] B. afzelii may not be the exclusive etiologic agent of ACA; Borrelia garinii has also been detected.[citation needed]

Presentation

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The rash caused by ACA is most evident on the extremities. It begins with an inflammatory stage with bluish red discoloration and cutaneous swelling, and concludes several months or years later with an atrophic phase. Sclerotic skin plaques may also develop.[citation needed] As ACA progresses the skin begins to wrinkle (atrophy).

Cause

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Diagnosis

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Generally a two-step approach is followed. First, a screening test involving IgM and IgG ELISA. If the ELISA screening has a positive or equivocal result, then the second step is to perform a Western Blot as a confirmatory test.

Other methods include microscopy and culture (in modified Kelly's medium) of skin biopsy or blood samples.

Treatment

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Antibiotics is recommended in treatment of ACA. Doxycycline is often used.[3][4] Resolution may take several months.[3] Skin damage and nerve damage may persist after treatment.[3]

History

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The first record of ACA was made in 1883 in Breslau, Germany, where a physician named Alfred Buchwald first delineated it.[citation needed]Herxheimer and Hartmann described it in 1902 as a "tissue paper like" cutaneous atrophy.

See also

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References

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  1. ^ Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 978-1-4160-2999-1.
  2. ^ James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: clinical Dermatology. Saunders Elsevier. ISBN 978-0-7216-2921-6.
  3. ^ a b c d Kullberg, Bart Jan; Vrijmoeth, Hedwig D.; van de Schoor, Freek; Hovius, Joppe W. (2020-05-26). "Lyme borreliosis: diagnosis and management". BMJ (Clinical Research Ed.). 369: m1041. doi:10.1136/bmj.m1041. ISSN 1756-1833. PMID 32457042. S2CID 218911807.
  4. ^ National Guideline Centre (UK) (2018). Evidence review for management of acrodermatitis chronica atrophicans: Lyme disease: diagnosis and management. NICE Evidence Reviews Collection. London: National Institute for Health and Care Excellence (NICE). ISBN 978-1-4731-2919-1. PMID 35201695.

Bibliography

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  • Stanek G & Strle F (2008) Lyme Disease—European Perspective| Infectious Disease Clinics of North America | Volume 22 | Issue 2 | June 2008, Pages 327-339|Abstract
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