Scleromyxedema is a specific type of dermatosis that usually presents in female patients. Scleromyxedema is characterized by pachyderma that appears over a large area and has papules on it. Generally, plasmacytomas manifest in the form of scleromyxedema. In this case, the disease expresses itself eruptively on the skin and often appears before hematologic phenomena.
What is scleromyxedema?
Scleromyxedema is also known by synonymous terms as lichen myxoedematosus and Arndt-Gottron syndrome. The latter name is derived from the physicians Gottron and Arndt, who first described the skin disorder in 1954. Basically, scleromyxedema is a special form of lichen myxoedematosus that is particularly severe. Thus, the characteristic lichenoid papules appear in the context of scleromyxedema. In addition, the skin thickens and hardens in the affected areas, with mucins deposited in certain skin layers. In addition, there is a marked increase in fibrosis on the skin. Scleromyxedema usually results from monoclonal paraproteinemia, with either the IgG or IgG1 type present.
Causes
The exact causes of scleromyxedema are currently unknown. Only certain mechanisms of the development of scleromyxedema have been explored. First, a plasmacytoma is present, which significantly increases fibroblast activity. As a consequence, increased mucins are deposited in the skin. In principle, from the point of view of some physicians, scleromyxedema is a special variant of lichen myxoedematosus. The fibroblasts cause the skin to produce and store increased amounts of mucopolysaccharides with an acidic pH. In this process, a special factor in the serum activates the fibroblasts. So far, the exact role of paraproteinemia in the development of scleromyxedema has not been clarified. Basically, fibroblasts remain in a stimulated state even when paraproteinemia is no longer present. In addition, the proportion of paraproteins does not provide an indication of the severity of scleromyxedema.
Symptoms, complaints, and signs
Scleromyxedema is characterized by three main typical symptoms that facilitate diagnosis of the skin disease. For example, patients suffer from thick and wrinkled skin resembling that of elephants. The affected skin areas are hyperpigmented and are considered pachydermic. In addition, individuals affected by scleromyxedema exhibit skin florescences resembling scleroderma. In addition, mimic rigidity is present. Finally, the typical papules appear on the skin of the affected individuals, which are close together, lichenoid, and resemble the natural color of the skin. The papules cause itching in numerous cases and are sometimes arranged in lines. In scleromyxedema, these papules occur primarily on the neck and face. Scleromyxedema develops in the majority of patients between the fourth and sixth decades of life. Most often, scleromyxedema appears on the neck, glabella, arms, hands, and trunk. In addition, symptoms also appear on the internal organs. The arteries of the corona as well as the kidney sclerose, so that corresponding complications follow. Sometimes the lungs are involved in the disease, with pulmonary fibrosis developing after several years. Impairment of esophageal motility is also possible, as are polyneuropathies and arthritides. In rare cases, dermato-neuro syndrome develops as a complication in the setting of scleromyxedema.
Diagnosis and course of the disease
Diagnosis of scleromyxedema is made by an appropriate specialist. During the history taking, he or she discusses the patient’s medical history as well as possible chronic diseases and similar complaints in close relatives. The person suffering from scleromyxedema describes the symptoms and the circumstances of the initial manifestation. Finally, the attending specialist uses various clinical examination methods to diagnose scleromyxedema. First, analyses of the blood are usually used. Then, the specialist takes tissue samples from the areas of the skin affected by scleromyxedema and orders an examination in the laboratory. The excessive accumulation of mucins in the skin can be detected. The physician also detects fibrosis and degraded connective tissue fibers.When diagnosing scleromyxedema, the physician has to perform an extensive differential diagnosis, since the symptoms of the disease also occur in a similar combination in numerous other diseases. For example, the physician excludes dome necrosis, systemic scleroderma, acrosclerosis, and Raynaud’s symptomatology. Circumscribed disseminated scleroderma, systemic nephrogenic fibrosis, and scleroedema adultorum should also be distinguished from scleromyxedema. Finally, pretibial myxedema, eosinophilic fasciitis, and mucinoses with other causes also warrant consideration in the differential diagnosis.
Complications
If scleromyxedema is not treated, additional complications usually develop during its course. Skin lesions such as abscesses and papules are typical of the disease and are associated with pain and itching and can leave scars if treated incorrectly or too late. If the disease spreads to the heart and kidney arteries, serious complications such as kidney failure and cardiac arrhythmias can develop. Since the noticeable skin changes typically occur on the face and neck, sufferers often also perceive this as an aesthetic flaw. Mental distress such as social anxiety and inferiority complexes may develop, which in turn are associated with complications. As a late consequence of scleromyxedema, pulmonary fibrosis is seen in individual cases. Impairments of the esophagus, disorders of the peripheral nervous system, and wear and tear diseases of the joints may also occur. Rarely, dermato-neuro syndrome develops as a result of dermatosis. Complications can also occur in the course of radiation therapy. In addition to the typical radiation hangover, which is associated with headaches, nausea and vomiting, late effects such as changes in the genetic material and degeneration of tissue can also occur. In children, growth disorders may occur in isolated cases.
When should you see a doctor?
Scleromyxedema usually always requires medical treatment. It is a serious disease of the skin, which must be examined and treated by a doctor in any case. Therefore, in order to prevent further spread of the disease, early diagnosis is also very important. A doctor should be consulted in case of scleromyxedema when papules and pustules appear. These can appear on the entire body and do not disappear on their own. Furthermore, a strong itching on the skin indicates scleromyxedema and must be checked by a doctor if it does not disappear on its own. In many cases, the kidneys and lungs of the affected person also suffer from scleromyxedema, so that severe discomfort can occur in these organs. In this case, too, a doctor must be consulted to prevent further complications, since self-healing cannot occur with this disease. In the case of scleromyxedema, a general practitioner or a dermatologist should be consulted in the first place. The disease can be treated relatively well so that no further complications occur.
Treatment and therapy
The measures and possible success of the treatment of scleromyxedema depend mainly on the severity of the dermatosis as well as the therapy of the plasmacytoma present. A causative method of treatment of scleromyxedema is not currently available. Symptoms may be relievable by means of retinoids as well as radiation therapy with PUVA rays.
Prevention
It is not yet known exactly how scleromyxedema can be effectively prevented. For this purpose, the causes of the skin disease have not yet been sufficiently researched. Therefore, it is particularly important for patients with scleromyxedema to see a doctor immediately if they experience the typical main symptoms of the dermatosis in order to prevent late effects of the disease, for example on the internal organs.
Aftercare
After treatment of scleromyxedema, the aftercare phase begins. How this proceeds depends on the type and severity of the skin problem. To date, there is no known effective prevention. This makes it so important for patients to monitor their skin and make a doctor’s appointment quickly if they notice any irregularities. A short-term response can prevent worse consequences of the disease. If, following treatment, the skin’s appearance again shows abnormalities, those affected can readjust their skin care products. The doctor will be happy to help them with this.With the renunciation of perfumed creams and cosmetics, the risk of a relapse decreases. In everyday life, it is possible to consciously reduce the stress on the skin. Products containing alcohol or aggressive substances can irritate the skin surface. Instead, natural-based products recommended by the attending physician or even the pharmacist can be used. In addition to protecting the skin, psychological stability plays a role. Skin irritation can weaken self-confidence and thus negatively affect the quality of life. Participation in a self-help course is very helpful in this context and counteracts inferiority complexes. Once patients have come to terms with their condition, it is easy for them to deal with unpleasant situations and remain relaxed.
Here’s what you can do yourself
The options for self-help are very limited in the case of scleromyxedema. Since the cause of the disease has not yet been fully clarified, it has not yet been possible to identify sufficient therapeutic methods or supportive approaches. Therefore, it is important to seek the cooperation of a physician already at the first irregularities of the skin appearance. In addition, the creams and skin care products used should be coordinated with the attending physician. To avoid additional stress or irritation of the skin, perfumed cosmetics should not be used. It is also important to ensure that the skin care products do not contain alcohol or other aggressive ingredients. It is recommended to use cosmetic products specially made for the needs of the skin. Psychological stability is important in dealing with the disease. Therefore, self-confidence should be supported and an open approach to the disease is advisable. This approach can help to prevent unpleasant situations such as glances or questions in everyday life. Wearing clothes or using accessories can be designed in such a way that large parts of the skin are protected. In addition, the clothing worn should also be tailored to the needs of the skin. Therefore, the composition of the manufactured clothing should be checked before wearing.
