Acrodermatitis Chronica Atrophicans Herxheimer: Causes, Symptoms & Treatment

Acrodermatitis chronica atrophicans Herxheimer is a disease of the skin. In the majority of cases, the disease progresses chronically and, due to its typical symptoms, fulfills the dermatological model of the final stage of so-called Lyme disease. Acrodermatitis chronica atrophicans Herxheimer is counted among the atrophic skin diseases.

What is acrodermatitis chronica atrophicans Herxheimer?

The first symptoms of acrodermatitis chronica atrophicans Herxheimer develop a few weeks to several years after the first infection with Borrelia burgdorferi. Acrodermatitis chronica atrophicans Herxheimer received its name in reference to a German dermatologist, Doctor Karl Herxheimer. In some cases, the disease is also called dermatitis atrophicans chronica progressiva or acrodermatitis chronica atrophicans. Sometimes it is also called Herxheimer’s disease for short. Basically, acrodermatitis atrophicans Herxheimer is a disease of the skin, which in some cases appears in the final stage or the third stage of Lyme disease. Acrodermatitis chronica atrophicans Herxheimer occurs primarily in Europe, but is also found in the United States and parts of Asia. In the majority of cases, the disease affects young adults and older females. In the Asian and European regions, a link is seen between acrodermatitis chronica atrophicans Herxheimer and Borrelia afzelii.

Causes

According to the current state of medical research, the cause of acrodermatitis chronica atrophicans Herxheimer is primarily seen in a specific pathogen. This is Borrelia afzelii. This pathogen belongs to the so-called Borrelia and occurs predominantly in the European region. For this reason, Borrelia afzelii often affects people who suffer from Lyme disease. Infection with Lyme disease pathogens threatens acrodermatitis chronica atrophicans Herxheimer in the late stages of the disease.

Symptoms, complaints and signs

Acrodermatitis chronica atrophicans Herxheimer is characterized by several typical symptoms and complaints that indicate the disease. However, the signs differ from patient to patient in terms of their individual appearance and severity. Basically, acrodermatitis chronica atrophicans Herxheimer is characterized by a progressive course in the majority of cases. Mostly, the disease appears in the area of the limbs and here especially on the extensor sides on the legs. At an early stage, acrodermatitis chronica atrophicans Herxheimer appears in the form of swelling as well as a livid discoloration of the skin. Further symptoms do not usually appear. In this case, lymphocytic infiltrations can be detected in histopathological examinations. In the later course of the disease, the subcutaneous tissue of the skin loses substance, which is also called atrophy. As a result, the veins under the skin show up very clearly as they protrude more. Overall, the skin makes a very thin impression, which is why the term cigarette paper skin is also used in this context. In addition, discoloration of the affected areas of the skin occurs. Basically, the first symptoms of acrodermatitis chronica atrophicans Herxheimer develop a few weeks to several years after the first infection with Borrelia burgdorferi. Thus, the time span is relatively long and numerous patients do not remember the causative tick bite. The swelling on the skin usually only appears on one leg and has a dark purple color. However, there are usually no other symptoms at the beginning of the disease. Later, blood vessels shimmer under skin and skin around joints develops fibromatous thickening. An accompanying symptom of acrodermatitis chronica atrophicans Herxheimer is very often oligoarthritis or so-called monarthritis, which occurs in the area of large joints. The knee joint is often affected. In addition, the nerves are sometimes involved in the form of radiculoneuritis or axonal polyneuropathy. Tertiary neuroborreliosis is a potential complication. If nerves in the subcutaneous area are affected, localized disturbances of sensibility may occur.

Diagnosis and course

The diagnosis of acrodermatitis chronica atrophicans Herxheimer should always be made by an appropriate specialist. As part of making the diagnosis, the attending physician first takes a thorough medical history. In doing so, he discusses the patient’s medical history, possible previous illnesses and any family history of health complaints with the patient. This is followed by various clinical examinations. In the case of acrodermatitis chronica atrophicans Herxheimer, tests are performed to determine whether the patient has Lyme disease. It is also possible to perform a skin biopsy. A thorough differential diagnosis is particularly important. Diseases to be clarified include erysipelas or erysipelas carcinomatosum, acrocyanosis, and eosinophilic fasciitis. In addition, the patient should be evaluated for lymphoplasmocytoid immunocytoma as well as erythromelalgia. Other potential diseases include scleroderma as well as linear morphaea and scleroedema adultorum Buschke. Finally, arthritis urica should be ruled out, which sometimes manifests in similar symptoms.

Complications

Acrodermatitis chronica atrophicans Herxheimer is a chronic skin disease that is more common in Europe than the United States or Asia. It affects young adults as well as older women. The pathogenesis is assumed to be caused by Borrelia bacteria. The first symptoms appear either within a few weeks or several years after an infection with Borrelia pathogens. Clearly recognizable is a swelling of the skin on the leg with a dark purple color. Already after a short time the skin becomes visibly thinner and the blood vessels as well as veins shine through. This is accompanied by the development of knee joint inflammation. In addition, there are fibroses on the joints. If the accompanying symptoms are not medically clarified in time, the course of the disease becomes more complicated. This leads to chronic forms of joint arthritis, an inflammatory disease of the nerve roots of the spinal cord and the peripheral nervous system. As a consequence, severe sensory disturbances, fever attacks and tearing headaches are imminent. If the accompanying arthritic symptoms are already pronounced, arthroscopic ablation of the diseased synovial membrane is performed. The procedure can cause further complications and requires a long healing process as well as independent medical treatment. Differential diagnosis can clarify whether acrodermatitis chronica atrophicans Herxheimer due to Lyme disease is present. A skin biopsy may also be performed depending on the severity of the case. If the symptom is not yet atrophic, high-dose antibiotics are given. Otherwise, the disease is no longer considered treatable with medication.

When should one go to the doctor?

Herxheimer’s acrodermatitis chronica atrophicans must be treated by a doctor. As a rule, this disease does not progress positively on its own, so treatment by a medical professional must be initiated in any case. As a rule, the doctor should be consulted when there are various complaints on the skin, which in most cases occur without any particular reason. In most cases, those affected by acrodermatitis chronica atrophicans Herxheimer suffer from swelling or itching, which can spread to various regions of the body. Furthermore, it is not uncommon to experience disturbances in sensitivity and paralysis of certain areas. If these symptoms occur, a medical professional must be consulted immediately. As a rule, acrodermatitis chronica atrophicans Herxheimer should be treated by a dermatologist. In this case, the treatment is carried out with the help of medication and in most cases leads to success.

Treatment and therapy

At the beginning of acrodermatitis chronica atrophicans Herxheimer, penicillin G is administered in high doses. In addition to penicillin, administration of cefotaxime is also possible, both orally and as an infusion.

Outlook and prognosis

Acrodermatitis chronica atrophicans Herxheimer causes a number of different symptoms. Those affected suffer from severe swelling and discoloration of the affected regions. Furthermore, pain may also occur, which can considerably reduce the quality of life. The skin appears very thin and can show bleeding due to simple external influences.In many cases, the symptoms of acrodermatitis chronica atrophicans Herxheimer appear only at the end of the disease, so that the disease is not directly associated with a tick bite. Likewise, paralysis and severe disturbances of sensitivity can occur, which not infrequently can lead to psychological discomfort or even depression in the patient. Furthermore, the disease can also lead to inflammation of the knee joints, which can result in walking difficulties. The affected person may then be dependent on the use of walking aids. Sufferers also suffer from severe fever and headaches. The patient’s ability to cope with stress decreases enormously. As a rule, acrodermatitis chronica atrophicans Herxheimer can be treated relatively easily with the help of antibiotics. This resolves all the symptoms. Life expectancy is also not reduced by this disease.

Prevention

Acrodermatitis chronica atrophicans Herxheimer can not be prevented in every case. Tick bites are unavoidable to some extent. One possibility is vaccination against Lyme disease.

Aftercare

As a rule, the options for aftercare in acrodermatitis chronica atrophicans Herxheimer are relatively limited. The patient is always dependent on medical treatment for this disease, and self-healing does not occur. Since the disease is also contagious, contact with other people should be prevented until the complaints and symptoms of acrodermatitis chronica atrophicans Herxheimer have subsided. Since the disease is usually treated with the help of medications, the affected person must take them regularly, and the possible interactions with other medications should also be taken into account. When taking antibiotics, it should also be noted that they should not be taken together with alcohol, as this reduces their effect. Likewise, parents should pay attention to regular intake in children. Since acrodermatitis chronica atrophicans Herxheimer can also lead to psychological discomfort or severe depression, the affected person should talk to his friends or family about the symptoms. In severe cases, however, a visit to a psychologist is necessary. Furthermore, contact with other sufferers of acrodermatitis chronica atrophicans Herxheimer may also prove useful, as this can lead to an exchange of information.

What you can do yourself

Despite all efforts, acrodermatitis chronica atrophicans Herxheimer is a disease with a chronic course. Therefore, in the context of self-help, there are no sufficient options that will bring about a cure for the affected person. Supportive measures can be taken to promote general well-being, the success of which must be evaluated on an individual basis. If, in the further course of the disease, there are increasing changes in the skin’s appearance, the physician’s instructions should always be followed. Acrodermatitis chronica atrophicans Herxheimer occurs in the final stage of Lyme disease. Therefore, in normal cases, medical care is already taking place and should not be interrupted. In addition, the patient can promote health through his lifestyle. A balanced and healthy diet strengthens the body’s immune system. This helps in dealing with pathogens and promotes recovery. The use of cosmetic products should be avoided whenever possible. Unless they promote the healing process, the ingredients of cosmetics can slow it down in unfavorable cases. Clothing should not be made of synthetic materials. Daily change of clothes is necessary and sweat should be well absorbed by the worn fabrics. If psychological irregularities occur due to the visual changes, the choice of clothing can cover large areas of the body well.