Akrodermatitis chronica atrophicans Herxheimer
| Classification according to ICD-10 | |
|---|---|
| L90.4 | Akrodermatitis chronica atrophicans Herxheimer's disease |
| ICD-10 online (WHO version 2019) | |
The acrodermatitis chronica atrophicans or acrodermatitis chronic atrophic in short, ACA 's as chronic progressive disease, the dermatological model of the third stage (final stage) of Lyme disease .
In the ICD-10 it is classified as L90.4 under " Atrophic skin diseases ". It was named after Karl Herxheimer (1861–1942), a German dermatologist .
Synonyms
- Herxheimer's disease
- Acrodermatitis chronica atrophicans
- Dermatitis atrophicans chronica progressiva
- Atrophia cutis idiopathica
- Herxheimer's disease
Epidemiology
The disease occurs mainly in Europe, but also in the USA and Asia, and mostly affects people of young to middle adulthood and older women. In Europe and Asia, the disease is also associated with Borrelia afzelii .
Symptoms
Months to years (weeks to decades) after the initial infection with the Borrelia burgdorferi pathogen - few patients remember the responsible tick bite - usually an edematous , darkly vivid (i.e. dark bluish) develops on one of the legs (the extensor side of the distal extremities ) Swelling of the skin that causes little discomfort. After months, this infiltrative stage is replaced by a progressive reduction ( atrophy ) of the subcutaneous fatty tissue (the subcutis ) and a decrease in skin thickness, so that the skin vessels begin to shimmer through. In addition, the skin can thicken fibromatous over the joints , so the fiber content of the connective tissue of the skin increases.
In many cases, accompanying symptoms are oligoarthritis ( monarthritis ) of the large joints (or a large joint, often the knee joint ); involvement of the nerves ( radiculoneuritis , axonal polyneuropathy ; a complication is the development of tertiary neuroborreliosis ).
Differential diagnosis
- Erysipelas , erysipeloid , Erysipelas carcinomatosum
- Lymphoplasmocytoid immunocytoma
- Acrocyanosis
- Erythromelalgia
- Eosinophilic fasciitis
- linear morphea , disseminated circumscribed scleroderma, progressive scleroderma (see scleroderma )
- Scleroedema adultorum Buschke
- Corticosteroid action
- Cutis marmorata (marble skin)
- Telangiectasia
With regard to joint symptoms, one should be considered
treatment
Therapy is carried out with high-dose antibiotics as an infusion ( penicillin , cefotaxime ) or orally ( tetracyclines ). In the atrophic stage, however, the disease is “burned out” and no longer treatable with medication.
Treatment of the accompanying symptom arthritis: NSAIDs , corticosteroids only after pretreatment with antibiotics, synovectomy
