2.2.10 Acrodermatitis Chronica Atrophicans
Grading & Level of Importance: B
Lyme borreliosis; Late cutaneous Lyme borreliosis; Herxheimer's disease.
In the European population < 10 : 100.000 pro year. In the USA rare, in northern Mexico some infections. All ages can be involved, but adults preferred. Individuals at special risk: farmers, joggers, hikers, dog owners, forest workers.
Cutaneous manifestation of chronic Borrelia burgdorferi sensu latu species B. afzelii infection leading in a first step to inflammatory edematous changes and in a later step to irreversible atrophy. Concurrent neurological and /or cardiac involvement are common.
Aetiology & Pathogenesis
Late manifestation of chronic infection with almost the spirochaete Borrelia afzelii. Transfer of borrelia bacteria mostly via tick sting (Ixodes ricinus). History of insertion of hypostoma into the skin and sampling of blood and in parallel introduction of tick saliva and content of intestinal fluids with borrelia mostly 2 - 6 months back. Strong B-cell activation and accumulation of plasma cells in situ. Persistence of microbes over decades possible. Destruction of dermal tissue, nerves and epidermal atrophy.
Signs & Symptoms
Stage I: inflammatory-edematous stage.
Stage II: atrophic stage (cigarette paper skin, telangiectases, pigmentary changes). Sclerosis (scleroderma-like collagen changes, ulnar and tibial bands) and hard fibrous nodules (up to several cm, cartilage-like nodules about joints). Associated allodynia and axonal peripheral neuropathy.
Hands and feet, ellbows, knees, initially unilateral, later symmetrical. Unusual localization possible.
Early inflammatory and late atrophic stage.
Laboratory & other workups
Sedimentation rate elevated, borrelia IgG titer raised, sometimes IgM persisting. Typical Outer surface proteins Osp 17 (p21), Osp A and B, p 30,43,45,58 83,100 persisting antigens.
Atrophy of epidermis. Infiltrate rich in plasma cells and additionally lymphomononuclear cells. No neutrophils or eosinophils. Collagen and elastic fiber destruction. Often adnexal structures with follicles and sweat glands are missing. Sometimes plasma cells around nerve bundles.
Chronic over years.
Soft tissue and muscle atrophy. Polyneuropathy. Increased vulnerability of atrophic skin.
Clinical features, histology, PCR, borrelia serology positive with antigen subtype profiling necessary.
Prevention & Therapy
Prevention of reinfection to tick stings. Treatment according to stage related guideline. First choice systemic antibiotic is doxycycline orally 3 weeks 200mg / day. Infusions with ceftriaxone.
Often patients consider suffering from chronic borreliosis of different organ manifestations. Huge amount of misleading web informations existing.
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