Acrodermatitis chronica atrophicans

• 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.

• 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.