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What does the abbreviation Bazex Syndrome stand for?

When bazex syndrome is a disease of the skin. Bazex syndrome occurs rarely and is one of the paraneoplastic diseases associated with cornification disorders (medical term acrokeratosis). Bazex syndrome occurs in connection with carcinomas that are localized in the area of ​​the upper respiratory tract and the esophagus. Bazex syndrome can also develop with cervical metastases of the lymph nodes.

What is Bazex Syndrome?

The Bazex syndrome is synonymous by some doctors as paraneopastic acrokeratosis or acrokeratosis type Bazex. The concept of illness is derived from the person who first described the illness. The dermatologist Bazex scientifically described Bazex syndrome for the first time in 1965. Attention should be paid to the risk of confusing the Bazex syndrome with the Bazex-Dupre-Christol syndrome.

Bazex syndrome has an estimated prevalence of 1: 1,100,000. Thus, the Bazex syndrome occurs extremely rarely and has only been diagnosed by a doctor in around 150 people since it was first described. Statistical analyzes show an increased incidence of Bazex syndrome in males after the fourth decade of life. The average age at first onset is 61 years.

Bazex syndrome often develops on the hands, fingers, nails, feet and ears. Typical skin changes are formed in a symmetrical form. Bazex syndrome is characterized by psoriasis-like erythema (psoriasis) and in some cases it can spread to the legs, arms, or trunk.

Causes

The exact causes of the Bazex syndrome have not been sufficiently researched to make reliable statements about the origins of the syndrome. Some researchers suspect that increased skin growth as a result of the carcinoma may be behind the development of the skin changes. In addition, some medical professionals consider cross-reactivity between tumor antigens and epidermal structures.

Symptoms, ailments & signs

The Bazex syndrome shows itself in typical lesions of the skin that are reminiscent of psoriasis and have a largely symmetrical shape. The rashes focus on specific areas of the body, for example hands, feet and ears. The skin changes mostly develop without any further symptoms.

In the case of histological analyzes of the skin lesions, the results are often rather unspecific and indicate, for example, hyperkeratosis in connection with parakeratosis. In many patients, Bazex syndrome is diagnosed before the cancer itself. This is often a squamous cell carcinoma of the airways and esophagus.

The disease usually starts in the ear area. In addition, the characteristic erythema often develops on the bridge of the nose. About a fifth of patients suffer from itching of the skin from Bazex syndrome. In addition, onychodystrophy, subungual hyperkeratosis and palmar hyperkeratosis often occur. In any case, Bazex syndrome is associated with certain carcinomas or metastases of the lymph nodes.

Sometimes the Bazex syndrome manifests itself as the first symptom of malignant tumors, so that it partly speeds up their diagnosis. The anomalies of the skin associated with the Bazex syndrome regress with successful treatment of the carcinoma.

Diagnosis & course

The diagnosis of Bazex syndrome begins with the anamnesis, which mainly relates to the symptoms and the living conditions of the patient. The general practitioner is suitable as the first point of contact, but usually refers the person suffering from Bazex syndrome to a dermatologist. There, the patient interview is followed by special examinations of the lesions on the skin. The specialist uses magnifying glasses to examine the changed areas of the skin and takes swabs from the diseased areas, which are then analyzed in the laboratory.

This makes it possible to diagnose Bazex syndrome. In the differential diagnosis, for example, psoriasis, mycosis fungoides, cutaneous cutaneous lupus erythematosus, dermatomyositis and photosensitivity must be ruled out. The diagnosis of Bazex syndrome implies a subsequent thorough examination of the airways and esophagus. In many cases, the doctor discovers the causative carcinoma.

When should you go to the doctor?

Since Bazex syndrome does not usually heal itself, a doctor must always be consulted. In most cases, those affected need treatment if they experience skin problems. This can lead to redness or itching. It is also not uncommon for breathing difficulties or other lung complaints to occur. Therefore, if breathing problems or unusual symptoms occur on the skin, an examination must be carried out by a doctor. As a rule, this can be done by a dermatologist.

In acute and urgent emergencies with Bazex syndrome, the hospital can also be contacted. In addition, psoriasis can occur on the skin with Bazex syndrome, and all regions of the body can be affected. For this reason, a doctor should also be consulted with these complaints if they occur suddenly or for no particular reason. In most cases it is possible to remove the tumor so that there are no further complications or a reduced life expectancy for the patient.

Complications

Bazex syndrome belongs to the group of paraneoplastic skin diseases and therefore has characteristic skin lesions. The acutely occurring rash in a largely symmetrical form is concentrated on individual body regions such as ears, feet, hands and nails. In any case, Bazex syndrome is an accompanying symptom of certain metastases or carcinomas in the lymph nodes and is a first symptom that indicates malignant tumors.

Most patients have cancers that are localized in the upper airways and esophagus. This skin disease has been diagnosed by doctors in 150 patients since it was first described in 1965. Most of the patients are male and are in their fourth decade of life. They suffer from extensive or selective keratinization of the skin in connection with psoriasis and eczema.

In many cases, these skin complications are associated with Bowen’s disease. This is a carcinoma that belongs to the group of white skin cancer. This cancer occurs mostly as squamous cell carcinoma, which is the second leading cause of skin cancer. If the Bazex syndrome is recognized and treated in good time, there is a positive prognosis for the patient.

Symptomatic and causal treatment begins with the underlying cancer of the esophagus and upper respiratory tract. As soon as a therapeutic success is achieved, the characteristic skin changes recede. If left untreated, Bazex syndrome is fatal due to the spread of carcinomas and malignant tumors.

Treatment & Therapy

The Bazex syndrome is treated both causally and symptomatically. The treatment of the causes starts with the underlying carcinoma in the area of ​​the esophagus and airways. Many patients have squamous cell carcinomas in these areas.

As soon as their therapy shows success, the changes in the skin that are typical for Bazex syndrome also recede. At the same time, a renewed development of skin changes indicates recurrent carcinoma. Symptomatic therapeutic approaches for Bazex syndrome concentrate primarily on the administration of the active ingredient acitretin.

Patients receive this drug either individually or in combination with UVA phototherapy. If the treatment of the carcinoma is positive, the changes on the skin usually disappear gradually. On the other hand, the abnormalities on the nails sometimes no longer regress.

Outlook & forecast

The prognosis for Bazex syndrome is favorable with adequate medical care. Although the cause of the disease has not yet been adequately clarified, doctors have found various therapy methods and drugs that lead to symptom relief.

Without medical treatment, there are various supportive measures that have a very helpful influence on the course of the disease. They include a healthy and balanced diet, various remedies and relaxation methods. The reduction of stress is promoted and the organism is supplied with sufficient nutrients so that a stable immune system is built up. However, full recovery is seldom achieved using these methods. In most cases, additional drug treatment is needed to cure symptoms. This does not address the causes, but fights the sequelae of Bazex syndrome.

The patient experiences a regression of the symptoms within a few weeks and in the further course usually no symptoms. In rare cases there is an unfavorable course of the disease. Bazex syndrome can lead to the formation of metastases. Depending on the diagnosis of the cancer and the earliest possible start of treatment, there is a risk of complications, healing delays or a significant deterioration in the state of health. Without treatment, metastasis can be fatal for the patient.

Prevention

Direct measures for the prevention of the Bazex syndrome are so far neither known nor adequately tested. Basically, it is possible to prevent Bazex syndrome by preventing the development of the causative carcinoma. But their prevention has not yet been adequately researched either.

The origins of squamous cell carcinoma, for example, have only been partially clarified. While some risk factors are known, most of the pathogenesis is still unclear. The Bazex syndrome itself serves in numerous cases as an important indicator of the presence of a malignant carcinoma in the area of ​​the esophagus and the airways. Because the location of these cancers is well known, their diagnosis and treatment often quickly follow that of Bazex syndrome.

Aftercare

After the actual treatment of Bazex syndrome, those affected have the opportunity to take advantage of various aftercare programs. In addition to regular examinations and therapeutic discussions, this also includes measures to improve the quality of life. The carcinoma may have restricted mobility, which must be compensated for in physiotherapy.

Other consequences must also be dealt with and remedied through targeted steps. The attending doctors help the patient to cope with the illness. You can arrange contacts to cancer counseling centers, self-help groups and other contact persons. Nutritionists and sports groups are also involved in the therapy as part of the aftercare.

Since the Bazex syndrome often takes a positive course, no long-term follow-up measures are usually necessary. The transition between cancer treatment and follow-up care is fluid, although the follow-up must first be checked at shorter intervals. If there are no complications and there are no signs of relapse, the intervals can be extended.

One to two years after the illness, it makes sense to have an examination every year, provided that there are no symptoms. If you experience any unusual symptoms, you should consult a doctor as soon as possible. Regular clarification of unusual complaints is also necessary between follow-up appointments.

You can do that yourself

In addition to conventional medical treatment, some self-help measures, home remedies and alternative healing methods can also help with Bazex syndrome.

Initially, however, those affected should adopt a healthy and active lifestyle with sufficient exercise, a balanced diet and enough relaxation and sleep. Various diets also increase the general well-being and improve the complexion, which is usually badly affected in Bazex syndrome.

For a better complexion, care products with aloe vera, chamomile, witch hazel or St. John’s wort can also be used in consultation with the doctor. At the same time, the visual changes can be concealed by choosing suitable clothing or with the help of make-up. Adequate personal hygiene helps against possible inflammation and eczema on the skin.

In the long term, however, the disease that underlies Bazex syndrome must be treated. In order for this to work, those affected should primarily follow the advice and tips of the doctor. Additional measures such as visiting a self-help group or talking to a therapist can be carried out alongside this. It is advisable to discuss all self-help measures with the responsible doctor beforehand so that there are no complications later.

What does the abbreviation Bazex Syndrome stand for