Acrodermatitis chronica atrophicans
ACA; chronic atrophic acrodermatitis; Herxheimer disease
Acrodermatitis chronica atrophicans is the late-stage cutaneous manifestation of European Lyme borreliosis, caused predominantly by Borrelia afzelii. It develops months to years (median 5–10 years) after untreated or undiagnosed early Lyme disease, presenting on the extensor surfaces of distal limbs as initially erythematous-violaceous swelling that progresses to characteristic cigarette-paper atrophy with prominent underlying veins. Skin-oncology relevance is as a mimic of DLE, scleroderma, solar elastosis, lipodermatosclerosis and chronic venous insufficiency — frequently misdiagnosed as solar ageing in older patients in endemic regions. ACA is highly suggestive of Borrelia infection (specific serology positive); doxycycline / amoxicillin / ceftriaxone treatment halts progression but established atrophy is irreversible.
Clinical features
- Two phases:
- Inflammatory / oedematous phase — initial unilateral or bilateral violaceous-red infiltrated swelling on the extensor surfaces of distal limbs (dorsa of hands, feet, knees, elbows). Lasts months to years.
- Atrophic phase — progressive thinning with cigarette-paper texture; prominent underlying veins; loss of hair; sclerodermatous induration adjacent to joints ("fibrotic bands" / juxta-articular nodules).
- Distribution — almost always limbs; rarely trunk or face.
- Median age 50–70; female predominance.
- Endemic UK / Northern European exposure.
- Concurrent peripheral neuropathy in 60% — sensory in affected limb; consequence of regional Borrelia infection.
Diagnosis
- Serology — Borrelia ELISA and Western blot are almost always strongly positive in ACA (in contrast to early EM where serology often negative); high-titre IgG with multiple bands.
- Skin biopsy — perivascular and band-like lymphoplasmacellular infiltrate, telangiectasia, epidermal atrophy, dermal sclerosis in late phase.
- Tissue PCR for Borrelia — variable yield; supportive when positive.
- Tick exposure history may be remote (years earlier); ask about countryside / forest exposure.
Differential
- Discoid lupus erythematosus — atrophic scarring plaques; photodistribution.
- Scleroderma / morphea — sclerotic; less atrophic.
- Lipodermatosclerosis — sclerotic; pretibial; venous insufficiency.
- Chronic venous insufficiency — sclerotic, hyperpigmented; less atrophic.
- Solar elastosis / actinic damage — diffuse photodistribution; less limb-specific.
- Atrophie blanche / livedoid vasculopathy — porcelain-white scars after ulceration.
Management
- Doxycycline 100 mg twice daily for 28 days (longer than for EM).
- Alternatives — amoxicillin 1 g three times daily for 28 days, ceftriaxone 2 g IV daily for 28 days (preferred for severe / neurological disease).
- Skin atrophy is irreversible — antibiotic treatment halts progression and treats associated peripheral neuropathy but does not reverse established cigarette-paper atrophy.
- Counsel patients about expectations.
- Photoprotection of atrophic skin.
- Surveillance for late cardiac / neurological Lyme manifestations.
References
- Stanek G et al. Lyme borreliosis. Lancet; 2012.
- NICE NG95. Lyme disease. London: NICE; 2018 (last updated 17 October 2018).
- Asbrink E, Hovmark A. Comments on the course and classification of Lyme borreliosis. Scand J Infect Dis; 1991.
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