Acrodermatitis chronica atrophicans

Last Updated: 2023-07-07

Author(s): Anzengruber F., Navarini A.

ICD11: 1C1G.14

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


  • Women aged 40 and over are more frequently affected.
  • Incidence (Europe): 10-130/100,000 per annum.
  • Rare outside Europe.

  • Exciter
    • Borrelia afzeli, less frequently B. burgdorferi, B. garinii.
  • Approximately 10% of all Borrelia infections develop ACA in the course of the disease. In only about 30% of cases, erythema migrans is remembered in the area of the affected localisation. A genetic predisposition may also play a role.

  • The course is divided into 3 stages:
    • Inflammatory-oedematous stage
    • Atrophic stage
    • Sclerotic stage

  • Inflammatory-oedematous stage
    • Edematous, blurred, livid macules and plaques, which spread distally and proximally
    • The so-called ulnar and tibial striae are ACA manifestations in the ventral region of the forearm and lower leg, respectively.
    • Typically, there is locoregional lymph node adenopathy.
  • Atrophic stage
    • In the course, there is the development of atrophic, cigarette-paper-like pleating. The underlying vascular networks are clearly visible and phlebectasia and
    • pigmentary shifts may occur.
    • In the course, loss of hair follicles and marked xerosis cutis or exsiccation eczema may occur.
  • Sclerotic stage
    • In the sclerotic stage, scleroderma-like skin changes can develop, especially on the lower legs and the backs of the feet
    • Hard, calcifying, skin-coloured to reddish nodules, especially in the elbow region (fibroid juxtaarticular nodules). Hernia-like skin symptoms are also described in the context of ACA.
  • Neuronal involvement
    • 40% of patients report "burning" pain and sensory irritation
    • Red. Muscle reflexes.
    • Muscle spasms.
    • Mild paresis.
    • Chronic fatigue syndrome.
    • Concentration disorders.

  • Associations
    • Spinocellular carcinoma
    • Circumscribed scleroderma
    • Lichen sclerosus et atrophicans
    • Pseudolymphomas of the skin
    • Lipomas
    • Fibromas
    • Malignant cutaneous B-cell lymphoma

  • History regarding tick bites, erythema migrans?
  • Clinical
  • γ-globulins are elevated
  • BSG is elevated
  • Borrelia serology (high antibody titres to Borrelia antigens (IgG)
  • In case of CNS symptoms, neurological presentation and, if necessary, CSF puncture or MRI

Over joints (ankles, knees, back of hands, elbows) and on the distal extremities extensorally. Sometimes, however, also proximal, on the trunk and facial.

  • Inflammatory-oedematous stage: orthokeratosis, dermal oedema, vascular dilatation, lymphohistiocytic infiltrate, which is mainly localised perivascularly and neurotropically.
  • Atrophic stage: epidermal atrophy, orthokeratosis, lymphocytic and plasma cell-rich infiltrate.

  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.