Bazex Syndrome: Causes, Symptoms & Treatment

Bazex syndrome is a disease of the skin. Bazex syndrome is rare and is one of the paraneoplastic diseases associated with disorders of keratinization (medical term acrokeratosis). Bazex syndrome occurs in association with carcinomas localized in the upper respiratory tract as well as the esophagus. Bazex syndrome also sometimes develops in cervical metastases to the lymph nodes.

What is Bazex syndrome?

Bazex syndrome is referred to synonymously by some physicians as paraneopastic acrokeratosis or acrokeratosis type Bazex. The disease term is derived from the initial describer of the disease. Bazex, a dermatologist, first scientifically described Bazex syndrome in 1965. Attention should be paid to the risk of confusion of Bazex syndrome with Bazex Dupre-Christol syndrome. Bazex syndrome occurs with an estimated prevalence of 1:1,100,000. Thus, Bazex syndrome is extremely rare and has only been medically diagnosed in approximately 150 people since it was first described. Statistical analyses show a clustered occurrence of Bazex syndrome in males after the fourth decade of life. The average age at first manifestation is 61 years. Bazex syndrome often develops on the hands, fingers, nails, feet and ears. Typical skin lesions form in a symmetrical pattern. Bazex syndrome is characterized by psoriasis-like erythema (psoriasis) and in some cases may extend to the legs, arms, or trunk.

Causes

The exact causes of Bazex syndrome have not been sufficiently researched to draw firm conclusions about its etiology. Some researchers suggest that increased growth of the skin as a result of carcinomas may be behind the development of the skin lesions. In addition, some physicians consider cross-reactivity of antigens of the tumor and epidermal structures.

Symptoms, complaints, and signs

Bazex syndrome manifests as typical lesions of the skin that resemble psoriasis and have a largely symmetrical shape. The exanthemas are concentrated on certain body regions, for example, hands, feet, and ears. The skin lesions predominantly form without further symptoms. Histologic analysis of the skin lesions often yields rather nonspecific results, indicating, for example, hyperkeratosis in association with parakeratosis. In many patients, Bazex syndrome is diagnosed before the actual carcinoma. This is often squamous cell carcinoma in the airway and esophagus. Usually, the disease starts in the area of the ears. In addition, the characteristic erythema often develops on the bridge of the nose. Approximately one fifth of patients suffer from itching of the skin due to Bazex syndrome. In addition, onychodystrophies, subungual hyperkeratoses and palmar hyperkeratoses often occur. In any case, Bazex syndrome is associated with certain carcinomas or metastases to the lymph nodes. Sometimes Bazex syndrome manifests as the first symptom of malignant tumors, so it sometimes hastens their diagnosis. The skin abnormalities associated with Bazex syndrome regress with successful therapy of the carcinomas.

Diagnosis and progression

The diagnosis of Bazex syndrome begins with the patient’s medical history, which is primarily concerned with the patient’s symptoms and circumstances. 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 altered areas of skin and takes swabs of the diseased areas, which are analyzed in the laboratory. This allows a diagnosis of Bazex syndrome to be made. In the differential diagnosis, psoriasis, mycosis fungoides, cutaneous cutaneous lupus erythematosus, dermatomyositis, and photosensitivity, for example, must be excluded. The diagnosis of Bazex syndrome implies a subsequent thorough examination of the airways and esophagus. In many cases, the physician discovers the causative carcinoma.

When should one go to the doctor?

Because Bazex syndrome does not usually heal itself, a doctor must always be consulted. In most cases, those affected are then in need of treatment when symptoms appear on the skin.This can lead to redness or itching. Likewise, it is not uncommon for breathing difficulties or other complaints to occur on the lungs. Therefore, if respiratory complaints or unusual symptoms occur on the skin, an examination by a physician must be performed. As a rule, this can be done at a dermatologist. In acute and urgent emergencies, the hospital can also be contacted for Bazex syndrome. Furthermore, Bazex syndrome can also cause psoriasis on the skin, and all regions of the body can be affected. For this reason, a doctor should also be consulted for these complaints if they occur without any particular reason or suddenly. In most cases, it is possible to remove the tumor, so there are no further complications and also no reduced life expectancy of the patient.

Complications

Bazex syndrome belongs to the group of paraneoplastic skin diseases and therefore has characteristic lesions of the skin. The acute rash, which is largely symmetrical, is concentrated in single body regions such as the ears, feet, hands, and nails. Bazex syndrome is in any case an accompanying symptom of certain metastases or carcinomas in the lymph nodes and a first symptom indicating malignant tumors. Most patients suffer from carcinomas localized in the upper respiratory tract and esophagus. This skin disease has been medically diagnosed in 150 patients since it was first described in 1965. Patients are predominantly male and are in their fourth decade of life. They suffer from extensive or intermittent keratinization of the skin associated with psoriasis and eczema. In many cases, these skin complications are associated with Bowen’s disease. This is a carcinoma that belongs to the white skin cancer group. This carcinoma usually occurs as squamous cell carcinoma, which is the second most common cause of skin cancer. If Bazex syndrome is detected and treated in time, there is a positive prognosis for patients. Symptomatic and causative treatment begins at the underlying carcinoma in the esophagus and upper respiratory tract. Once therapeutic success is achieved, the characteristic skin lesions regress. Without treatment, Bazex syndrome is fatal due to the spread of the carcinomas and malignant tumors.

Treatment and therapy

Bazex syndrome is treated both causally and symptomatically. Treatment of the causes starts with the underlying carcinoma in the esophageal and respiratory tract. In many patients, squamous cell carcinomas are present in these areas. As soon as their therapy is successful, the changes in the skin typical of Bazex syndrome also recede. At the same time, a reappearance of skin changes indicates recurrent carcinomas. Symptomatic therapy approaches of the Bazex syndrome concentrate mainly on the administration of the active substance acitretin. Patients receive this drug either individually or combined with UVA phototherapy. If the treatment of the carcinoma is positive, the changes on the skin usually disappear gradually. On the other hand, abnormalities on the nails sometimes do not regress.

Outlook and prognosis

The prognosis of Bazex syndrome is favorable with adequate medical care. Although the cause of the disease has not yet been adequately elucidated, physicians have found various therapeutic methods and medications 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. Stress reduction is promoted and the organism is supplied with sufficient nutrients so that a stable immune system is built up. However, complete recovery is rarely achieved when these methods are used. In most cases, additional drug treatment is needed to cure the symptoms. This does not address the causes, but combats the secondary symptoms of Bazex syndrome. The patient experiences a regression of the symptoms within a few weeks and in the further course usually freedom from symptoms. In rare cases, the course of the disease is unfavorable.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, delays in healing or a significant deterioration in health. Without treatment, the formation of metastases can have a fatal course for the patient.

Prevention

Direct measures to prevent Bazex syndrome are not yet known or adequately tested. In principle, prevention of Bazex syndrome is possible by preventing the development of the causative carcinomas. However, even their prevention has not yet been sufficiently researched. For example, the developmental background of squamous cell carcinomas is only partially understood. While some risk factors are known, most of the pathogenesis is still unclear. Bazex syndrome itself serves as an important indicator of the presence of malignant carcinoma in the esophagus and airways in numerous cases. Because the location of these carcinomas is well known, their diagnosis and treatment often quickly follow that of Bazex syndrome.

Follow-up

After the actual treatment of Bazex syndrome, there are various aftercare programs available to those affected. These include regular examinations and therapeutic discussions as well as measures to improve the quality of life. It is possible that the carcinoma has led to restrictions in the ability to move, which must be compensated for in the course of physiotherapy. Other consequences must also be dealt with and remedied through targeted steps. The attending physicians help the patient to come to terms with the disease. They can arrange contacts to cancer counseling centers, self-help groups and other contacts. Nutritionists and sports groups are also involved in the therapy as part of the aftercare. Because Bazex syndrome often has a positive course, long-term aftercare measures are usually not necessary. The transition between cancer treatment and aftercare is fluid, with the initial need to go for progress monitoring at shorter intervals. If no complications occur and there are no signs of relapse, the intervals can be extended. One to two years after the disease, a rotation of one examination per year is advisable, provided that no symptoms appear. If any unusual symptoms occur, a physician must be consulted as soon as possible. Regular clarification of unusual complaints is also necessary between follow-up appointments.

Here’s what you can do yourself

In addition to conventional medical treatment, some self-help measures, home remedies and alternative healing methods sometimes help with Bazex syndrome. First, however, a healthy and active lifestyle with sufficient exercise, a balanced diet and enough relaxation and sleep applies to those affected. Various diets additionally increase the general well-being and improve the skin appearance, which is usually severely affected by Bazex syndrome. For a better skin appearance, care products with aloe vera, chamomile, witch hazel or St. John’s wort can also be used in consultation with the doctor. Accompanying this, the optical changes can be concealed by choosing suitable clothing or with the help of make-up. Sufficient body hygiene helps against possible inflammation and eczema on the skin. In the long term, however, the disease underlying Bazex syndrome must be treated. For this to succeed, those affected should first and foremost follow the doctor’s advice and tips. Other measures such as visiting a self-help group or talking to a therapist can accompany this. It is advisable to discuss all self-help measures with the responsible physician beforehand so that complications do not arise later.