Acrodermatitis chronica atrophicans

Last Updated: 2020-11-19

Author(s): Anzengruber, Navarini

  • Taylor 1875
  • Buchwald 1883
  • Pick 1895
  • Herxheimer und Hartmann 1902

ACA.

  • Women over 40 years of age are more frequently affected.
  • Incidence (Europe): 10-130/100.000 per annum.
  • Rarely outside Europe.
  • Causative organism.
    • Borrelia-afzeli.
  • In about 10% of all Borrelia infections, an ACA develops in the course of time. In only about 30% of the cases an erythema migrans is memorable in the area of the affected localization. It is also possible that genetic predisposition plays a role.
  • Der Verlauf wird in 3 Stadien eingeteilt:
  • Inflammatory-edematous stage
  • Atrophic stage
  • Sclerotic stage
  • Inflammatory-edematous stage
    • Edematous, blurred, livid maculae and plaques spreading distally and proximally.
    • The so-called ulnar and tiba strips are ACA manifestations in the ventral region of the forearm or lower leg.
    • Typically, locoregional lymph node adenopathy occurs.
  • Atrophic stage
    • In the course of time, an atrophic, cigarette paper-like wrinkling develops. The underlying vascular nets are clearly visible and Phlebektasia and Pigment shifts may occur.
    • In the course of the disease, loss of hair follicles and pronounced xerosis cutis or desiccation eczema can occur.
  • Sclerotic stage
    • In the sclerotic stage, scleroderma-like skin changes can develop, especially on the lower leg and back of the foot.
    • Hard, calcifying, skin-colored to reddish knots, especially in the elbow region (fibroid juxta articular knots). Hernia-like skin symptoms are also described in the ACA.
  • Neuronal involvement
    • 40% of patients report "burning" pain and sensitive irritation.
    • Red. Muscle reflexes.
    • Muscle cramps.
    • Easy paresis.
    • Chronic fatigue syndrome.
    • Concentration problems.
    • Connotations
    • Spinocellular carcinoma.
    • Circumscriber scleroderma.
    • Lichen sclerosus et atrophicans.
    • Pseudolymphomas of the skin.
    • Lipomas.
    • Fibroms.
    • Malignant cutaneous B-cell lymphoma.
  • Medical history of tick bites, erythema migrans?
  • Clinic.
  • γ globulins are elevated.
  • ESR is elevated.
  • Borrelia serology (high antibody titers against Borrelia antigens (IgG)
  • In case of CNS symptoms neurological presentation and if necessary cerebrospinal puncture or MRI.
  • Inflammatory oedematous stage: orthokeratosis, dermal oedema, vascular dilatation, lymphohistiocytic infiltrate, which is mainly located perivascularly and neurotropically.
  • Atrophic stage: epidermal atrophy, orthokeratosis, lymphocytic and plasma-rich infiltrate.
Therapy after Lebwohl

Level of evidence
Doxycycline p.o. 100 mg 2x daily for 3 weeks C
Amoxicillin p.o. 500 mg 3x daily for 3 weeks C
Cefuroxim p.o. 500 mg 2x daily for 3 weeks D
  • Ceftriaxon IV 2g (max. 4g) 1x daily for 21 days.
  • Alternative: Doxycycline p.o. 100 mg 2x daily for 3-4 weeks.

 

Children/pregnancy:

  • Amoxicillin p.o. 50 mg/kg bw/d 2-3x daily
  • Alternative: Erythromycin p.o. or IV 500 mg 3-4x daily.
  • Alternative: Clarithromycin p.o. 15 mg/kg bw/day for up to 10 days.

 

  • The therapy should be repeated every 3 months, if necessary for 1 year or until the clinical findings have healed.
  • CAVE: Atrophies and sclerosing are not reversible. Antibody determinations have no therapeutic consequences.

 

  1. Herxheimer K , Hartmann K. Ueber Acrodermatitis chronica atrophicans. Arch f Dermat 1902;61:255-300.
  2. Müllegger RR, McHugh G, Ruthazer R, Binder B, Kerl H , Steere AC. Differential Expression of Cytokine mRNA in Skin Specimens from Patients with Erythema Migrans or Acrodermatitis Chronica Atrophicans. Journal of Investigative Dermatology 2000;115:1115-23.
  3. Sweitzer SE. ACRODERMATITIS CHRONICA ATROPHICANS. Arch Dermatol 1935;31:196.
  4. Lebwohl, Mark. Treatment of Skin Disease: Comprehensive Therapeutic Strategies. Elsevier, 2014. Print.