Acrodermatitis chronica atrophicans is a pathological skin change of an atrophic nature corresponding to the third stage of borreliosis. The first clinical symptom of the pathological process is erythema cyanosis, which develops against the background of unpleasant subjective sensations, minor skin soreness, arthralgia. Over time, atrophy phenomena occur in the area of the spot, the bluish hue of the skin is replaced by whitish, the epidermis and dermis thin out, begin to gather easily into folds, peel off. The diagnosis is made on the basis of anamnesis, clinic, histology data, RNIF, ELISA, PCR, immunoblotting. Treatment – antibiotic therapy, physiotherapy, vitamin therapy.
ICD 10
L90.4 Acrodermatitis chronica atrophicans
General information
Acrodermatitis chronica atrophicanss is a specific atrophic lesion of the skin, which is a distinctive feature of the third stage of infectious tick–borne borreliosis. For the first time, tick-borne infection caused by spirochete Borrelia burgdorferi became known in 1975, when an outbreak of this disease was registered and described in Connecticut (USA), however, acrodermatitis chronica atrophicans corresponding to the third stage of the disease was known long before thanks to the works of researcher Buchwald, who described the manifestations of this pathology back in 1883. In the middle of the twentieth century, due to the rapid development of serological diagnostics, the third stage of tick-borne infection, including its skin manifestations, was studied in detail. At this stage, acrodermatitis chronica atrophicans was associated with borreliosis.
The pathological process is widespread mainly in the countries of the Northern hemisphere, where there are natural reservoirs of tick-borne encephalitis. Acrodermatitis chronica atrophicans is more common in older women. Unlike borreliosis, which has seasonality, occurs at any time of the year. The urgency of the problem at the present stage is associated with an increase in the incidence of borreliosis and the need to confirm the cause of acrodermatitis chronica atrophicans in order to prescribe adequate and effective therapy.
Causes
The only reliably established causative agent of the disease is borrelia, transmitted to humans by the bite of the tick Ixodes ricinus. Some dermatologists believe that acrodermatitis chronica atrophicans can develop against the background of failures of the neuroendocrine system, although, most likely, these systemic disorders are the same manifestations of the third stage of Lyme disease as acrodermatitis chronica atrophicans itself.
Other authors attribute the disease to an autoimmune pathology that occurred when the body was infected with pathogens of syphilis, tuberculosis, and malaria. Injuries or the temperature-cold factor are sometimes considered as triggers of the pathological process. However, modern ideas about the mechanism of occurrence of acrodermatitis chronica atrophicans are still associated with borreliosis.
Pathogenesis
Cutaneous manifestations of the third stage of Lyme disease are a consequence of the damaging effect of circulating immune complexes (CEC). The development of acrodermatitis chronica atrophicans during the first two stages of borreliosis is latent, consists in the formation and accumulation of these complexes. After a tick bite, the spirochete enters the blood and lymph, spreads to all organs and tissues, occupies the lymph nodes. In them, the spirochete is able to live and reproduce for years.
Part of the borrelia circulating in the blood dies, releasing endotoxin into the bloodstream, stimulating the immune system. Antigen-antibody immune reactions occur, which result in the formation of circulating immune complexes formed after each antigen-antibody encounter.
CEC begin to have a negative effect on the dermis, disrupting its morphology, changing the permeability of blood vessels and forming inflammatory infiltrates. Neutrophils migrate from the blood to the area of the formed infiltrate. Live borrelia circulating in the blood stimulate the synthesis of interleukins, which exacerbates inflammation, contributing to a more intense destruction of the structure of the dermis.
One stage of borreliosis replaces another, new “portions” of spirochetes from the lymph nodes are constantly being thrown into the blood at this time. They secrete endotoxin again, which stimulates the formation of circulating immune complexes. This happens until the amount of CEC in the blood becomes sufficient for hyperstimulation of inflammation in the dermis, an additional change in the permeability of the capillary wall of the skin with visual skin manifestations. This occurs simultaneously with the transition of borreliosis to the third stage and indicates the chronization of the process.
Symptoms
A distinctive feature of acrodermatitis chronica atrophicans is its staging. There are several periods in the clinical picture of the pathological process.
- The first, erythematous-infiltrative (preatrophic) is characterized by the appearance of bright spontaneous erythema with a bluish tinge against the background of edematous skin, which eventually diffusely infiltrates, creating the basis for future atrophy. The skin manifestations are localized mainly on the extremities. Rashes are accompanied by prodromal sensations, a decrease in skin sensitivity, arthralgias, and a decrease in muscle contractility.
- The second, transitional (progressive-atrophic) period proceeds with an increase in exudative-inflammatory phenomena, which subsequently gradually subside. The skin loses moisture, flakes off, its elasticity decreases. It easily gathers into folds, becomes so thin that vessels begin to shine through it.
- The third period (stationary-atrophic) is characterized by the development of not only skin atrophy, but also dermatosclerosis. The skin turns brown, dyschromia occurs in the foci of acrodermatitis chronica atrophicans – hypopigmentation and apigmentation alternates with hyperpigmentation.
The process involves the appendages of the skin, the secretion of sebaceous and sweat glands is disrupted, downy hair falls out, nails are deformed. A distinctive feature of skin pathology in acrodermatitis chronica atrophicans is not only rashes, but also a violation of the tactile sensitivity of the skin with the preservation of the temperature and pain component.
There is also an atypical variant of acrodermatitis chronica atrophicans with unchanged skin sensitivity and immobility of the dermis in chronic foci of pathology by the type of scleroderma. Sometimes, with the development of the disease, dense periarticular subdermal nodes of different sizes with clear boundaries are formed, with a tendency to grouping. Small nodes (up to 5 cm in diameter) are flat, large ones (up to 30 cm in diameter) are spherical. The ability of such nodes to ulcerate is considered as a sign of their malignancy. Most often, the formation of nodes is combined with joint pathology.
Diagnostics
The clinical diagnosis is made by a dermatologist and an infectious disease specialist. The grounds for diagnosis are anamnesis, clinical picture and serological tests. Histology is connected to differentiate the pathological process. Consultations of narrow specialists are necessary to exclude systemic pathology.
INF (indirect immunofluorescence reaction) is used to determine the titer of borreliosis antibodies. ELISA (enzyme immunoassay) allows you to determine the presence of borrelia in the patient’s blood. PCR and immunoblotting make it possible to calculate the exact number of borrelia per unit volume of blood. Differentiate acrodermatitis chronica atrophicans with other types of atrophy and scleroderma.
Treatment
The effectiveness of therapy depends on the stage of the pathological process and the timeliness of the treatment initiated. Therapy of acrodermatitis chronica atrophicans is pathogenetic, it is carried out with courses of antibiotics, the total dose of which is calculated taking into account the results of serological testing and the weight of patients. Vitamin therapy and iron preparations are used simultaneously. If somatic pathology is detected, its correction is carried out.
With acrodermatitis chronica atrophicans, physiotherapy is effective: UVI, darsonval, galvanization, UHF, diathermy, hydrogen sulfide warm mineral baths, mud wraps, applications with ozokerite, paraffin, massage. Externally, ointments containing vitamins and vasoactive drugs are shown. After consultation with a surgeon, surgical removal of subcutaneous nodes is possible. The prognosis with adequate treatment is favorable.