Acrodermatitis chronica atrophicans L90.4

Author: Prof. Dr. med. Peter Altmeyer

Co-Autor: Fabian Müller

Our authors

Last updated on: 29.10.2020

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Synonym(s)

Acrodermatitis chronica atrophicans; Chronic cutaneous borreliosis; Dermatitis atrophicans chronica progressiva; Dermatitis atrophicans diffusa progressiva idiopathica Oppenheim; Herxheimer M.; Herxheimer's disease; Lyme borreliosis; Lyme disease; Pick-Herxheimer's disease; Taylor's disease; Ulna Strips

History
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Taylor, 1875; Buchwald, 1883; Pick, 1895; Hartmann and Herxheimer, 1902

Definition
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An eminently chronic (bacterial) disease caused by a Borrelia infection, leading to pronounced skin atrophy (cigarette-paper phenomenon) with extensive livid red erythema on the trunk and extremities.

Pathogen
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Especially caused by Borrelia afzelii.

Occurrence/Epidemiology
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Incidence (Europe): about 10/100.000 inhabitants/year. Common in Borrelia endemic areas, especially in some wooded areas of Austria, Poland and Eastern Europe.

Acrodermatitis chronica atrophicans is extremely rare in North America (the pathogen is B. afzelii, which is not common in America).

Manifestation
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About 10% of (untreated) Lyme Borrelia infections lead to acrodermatitis chronica atrophicans.

Occurrence is possible at any age, especially in the 5th-6th decade of life and more frequently in women (Strle F et al. 2013).

Occurrence usually months to years after infection in stage 3 of Lyme disease.

Localization
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Distal extremities (Strle F et al. 2013) and acra, often symmetrical.

Clinical features
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Infiltrative (edematous) stage: Months to years after the primary stage, circumscribed, rather discrete, at first diagnosis (when they are recognized as Borrelia infection) palm-sized, not very sharply defined, non-symptomatic, brownish-red, succulent, non-scalytic erythema and plaques are formed. These are often misinterpreted on the lower extremity as "chronic venous insufficiency". If left untreated, the lesions gradually spread to large, edematous, blurred, livid-red plaques with a shiny surface, often covering an entire body part and causing hardly any discomfort.

Atrophic stage: after months of existence, barely noticeable transition to a peculiarly shiny, thinned out, hairless (follicles are destroyed), atrophic skin stage. The brown-red discoloured skin is conspicuously wrinkled (and gives it its name), with clearly translucent vessels. The superficial veins run under this atrophic skin, as strangely attached winding strands, as they are otherwise only found in the withered skin of very old people. Often an allodynia, an exaggerated burning pain reaction to banal stimuli, is the result.

Optional side effects:

It is one of the inexplicable peculiarities of this disease that, in addition to the extensive atrophy of the skin and subcutis (in untreated condition), which characterizes the clinical picture, focal connective tissue growths, so-called fibroid juxtaarticular nodules form. These are possibly calcifying nodules as large as walnuts, especially over the elbows.

Furthermore:

  • Scleroderma-like, coarse, whitish shiny connective tissue plates, especially on the lower legs and back of the foot.
  • Secondary skin changes: loss of hair follicles, sebaceous and sweat glands, dry skin.
  • Tendency to desiccation eczema.
  • Torpid ulcerations due to minor traumas.
  • Increased incidence of pseudo-lymphomas of the skin, lipoma, fibroma, carcinoma, transition to malignant cutaneous B-cell lymphoma possible.
  • About 50% of infected persons suffer from axonal peripheral neuropathy (mostly lesionally limited)
  • A disseminated neuropathy is rarer.

Laboratory
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Strong increase of gamma globulins and BSG by stimulation of the B-lymphocyte system with maturation of plasma cells. High antibody titers against Borrelia antigens (IgG).

Histology
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Inflammatory oedematous stage: epithelium not very wide, orthokeratosis. Edema of the dermis with vascular dilatation. Characteristic are interstitial granulomatous infiltrates(CD68 positive) with thickened and homogenized collagen bundles and a banded infiltrate of CD4 positive T lymphocytes. Partially vacuolic degeneration of the basal cells. Plasma cells are not always detectable. In a larger study (Brandt FC et al. 2017) plasma cells were absent at 9 of 22 biospies. B. afzelii, serotype 2 is the preferred method for detection.

Atrophic stage: Epidermis atrophy with compact orthokeratosis; increasing loss of elastic fibres. Rather sparse, diffuse inflammatory infiltrate from lymphocytes and plasma cells.

Differential diagnosis
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  • Clinical differential diagnosis:
  • Histological differential diagnosis:
    • Lichen planus: prominent interface dermatitis with pronounced vacuolisation of the basal keratinocytes; no plasma cells.
    • Lichenoid drug reaction: usually marked dermal edema; often eosinophilia, no plasma cells.
    • Mycosis fungoides: typical halo formation around polymorphic lymphocytes, hardly any plasma cells, marked epidermotropy with vacuolated epidermis cells; eosinophilia is possible.
    • PLEVA: no histiocytes, no plasma cells.
    • Syphilitic exanthema: interface dermatitis with high infiltrate density, psoriasiform epidermal reaction, numerous plasma cells.
    • Atrophodermia idiopathica et progressiva: rather thin, lympho-histiocytic infiltrate, clumped collagen fibre bundles in the deep dermis; atrophic, hyperpigmented surface epithelium.

Internal therapy
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  • Therapy of the 1st choice is ceftriaxone (e.g. Rocephin) also for accompanying extracutaneous manifestations such as arthritis or CNS involvement (see Table 1). The antibiotic cycles should be repeated at 3-month intervals depending on the clinic and last 21-28 days due to the generation times.
  • Adults receive Ceftriaxone once/day 2 g i.v., in severe, refractory cases up to 4 g/day for 3 weeks. Children receive 50 mg/kg bw once/day up to a maximum dose of 2 g/day for 21 days. In premature and newborn infants up to 2 weeks, do not exceed doses of 50 mg/day/kg bw.
  • The duration of therapy depends on the clinical findings, if necessary the cycles must be repeated.
  • Antibody titre controls are only of limited use for the course of the healing process. However, a decrease in titer is observed under a sufficient therapy. Atrophy is not reversible.

Tables
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Therapy options for acrodermatitis chronica atrophicans

1st choice

Alternatively

Adults

Ceftriaxone once/day 2 g i.v. over 21 - 28 days

alternatively: cephalosporin of the III. gene (Cefotaxime) 3x2g i.v. over 21-28 days

Doxycycline 2 times/day 100 mg p.o. for 21-28 days or Tetracycline 2 g/day p.o. for 21 days

Children/pregnancy (danger of diaplacental transmission!)

Amoxicillin 50 mg/kg bw/day p.o. (2-3 times/day 500 mg/day p.o.) or Cefuroxime juice 20-30 mg/kg bw/day over 21 days

Erythromycin 3-4 times/day 500 mg p.o. or i.v. over 21 days

Literature
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  1. Abele DC, Anders KH (1990) The many faces and phases of borreliosis II J Am Acad Dermatol 23: 401-410
  2. Brandt FC et al (2015) Histopathology and immunophenotype of acrodermatitis chronica atrophicans correlated with ospA and ospC genotypes of Borrelia species. J Cutan Pathol 42:674-692.
  3. Brzonova I et al (2002) Acrodermatitis chronica atrophicans affecting all four limbs in an 11-year-old girl. Br J Dermatol 147: 375-378
  4. Buchwald A (1883) A case of diffuse idiopathic skin atrophy. Viertlj Schr Derm 10: 553-556
  5. Herxheimer K, Hartmann K (1902) About acrodermatitis chronica atrophicans. Arch Derm Syph 61: 57-76
  6. Hofmann et al.: S2k guideline Kutane Lyme disease. German Dermatological Society (DDG). Status March 2016, 6.3 Cutaneous late manifestations. accessed on: 17.November 2019.
  7. Leslie TA et al (1994) Acrodermatitis chronica atrophicans - a case report and review of the literature. Br J Derm 131: 687-693
  8. Lunemann JD et al (2003) Heterogeneity of Borrelia burgdorferi: etiopathogenetic relevance and clinical implications. Z Rheumatol 62: 148-154
  9. Moreno C et al (2003) Interstitial granulomatous dermatitis with histiocytic pseudorosettes: a new histopathologic pattern in cutaneous borreliosis. Detection of Borrelia burgdorferi DNA sequences by a highly sensitive PCR ELISA. J Am Acad Dermatol 48: 376-384
  10. Pick PJ (1900) Erythromellia. In: Commemorative publication Kaposi, Vienna, p. 915
  11. Stinco G et al(2014) Clinical features of 705 Borrelia burgdorferi seropositive patients in an endemic area of northern Italy. ScientificWorldJournal doi:10.1155/2014/414505.
  12. Strle F et al (2013) Gender disparity between cutaneous and non-cutaneous manifestations of Lyme borreliosis
    .PLoS One 8:e64110.
  13. Taylor RW (1875) On a rare case of idiopathic localized or partial atrophy of the skin. Arch Dermatol 2: 114-121

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Last updated on: 29.10.2020