Acrodermatitis Chronica Atrophicans

ICD-11

1C1G.14

Synonyms

Lyme borreliosis; Late cutaneous Lyme borreliosis; Herxheimer's disease.

Epidemiology

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.

Definition

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.

Localisation

Hands and feet, ellbows, knees, initially unilateral, later symmetrical. Unusual localization possible.

Classification

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.

Dermatopathology

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.

Course

Chronic over years.

Complications

Soft tissue and muscle atrophy. Polyneuropathy. Increased vulnerability of atrophic skin.

Diagnosis

Clinical features, histology, PCR, borrelia serology positive with antigen subtype profiling necessary.

Differential diagnosis

Thrombophlebitis, deep venous thrombosis, acrocyanosis, perniones, erysipelas, in stage I. Varicose veins in stage II. Sudeck's atrophy in both stages. Advanced aging of the skin with dermatoporosis.

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.

Special

Often patients consider suffering from chronic borreliosis of different organ manifestations. Huge amount of misleading web informations existing.

2.2.10 Acrodermatitis Chronica Atrophicans