Scleromyxedema is a special form of dermatosis that usually occurs in female patients. Scleromyxedema is characterized by an extensive pachydermia on which papules are located. In general, plasmacytomas manifest themselves in the form of scleromyxedema. The disease manifests itself eruptively on the skin and often occurs before hematological phenomena.
What is scleromyxedema?
The scleromyxedema is also known with synonymous terms as lichen myxoedematosus and Arndt-Gottron syndrome. The latter term is derived from the physicians Gottron and Arndt, who first described the skin disease in 1954. Basically, scleromyxedema is a special form of lichen myxoedematosus that is particularly difficult. For what does myopia mean, please visit etaizhou.info.
The characteristic lichenoid papules occur in the context of scleromyxedema. In addition, the skin on the affected areas thickens and hardens, with mucins being deposited in certain layers of the skin. In addition, there is clearly increased fibrosis on the skin. Scleromyxedema usually results from monoclonal paraproteinemia, with either the IgG or the IgG1 type being present.
The exact causes of scleromyxedema are currently unknown. Only certain mechanisms of the development of scleromyxedema have already been researched. First there is a plasmacytoma, which significantly increases the activity of the fibroblasts. As a result, more mucins accumulate in the skin.
In principle, from the point of view of some physicians, scleromyxedema is a special variant of lichen myxoedematosus. The fibroblasts ensure that the skin produces and stores more mucopolysaccharides with an acidic pH value. A special factor in the serum activates the fibroblasts.
It has not yet been clarified exactly what role paraproteinemia plays in the development of scleromyxedema. In principle, the fibroblasts remain in a stimulated state even when the paraproteinemia is no longer present. In addition, the proportion of paraproteins does not provide any indication of the severity of the scleromyxedema.
Symptoms, Ailments & Signs
Scleromyxedema is characterized by three typical main symptoms that make it easier to diagnose the skin disease. The patients suffer from thick and wrinkled skin reminiscent of that of elephants. The affected areas of skin are hyperpigmented and are considered pachydermia. In addition, people affected by scleromyxedema have skin lesions that resemble scleroderma.
There is also a facial rigidity. Finally, the typical papules appear on the skin of the patient, which lie close together, are lichenoid and resemble the natural color of the skin. In many cases, the papules cause itching and are sometimes arranged in a line. In scleromyxedema, these papules are primarily found on the neck and face.
Scleromyxedema develops in the majority of patients between the fourth and sixth decade of life. Scleromyxedema usually appears on the neck, glabella, arms, hands and trunk. In addition, there are also complaints in the internal organs. The coronary and renal arteries sclerose, resulting in complications.
The lungs are sometimes involved in the disease, with pulmonary fibrosis developing after several years. Impairments of the motility of the esophagus as well as polyneuropathies and arthritis are also possible. In rare cases, dermato-neuro-syndrome develops as a complication in the context of scleromyxedema.
Diagnosis & course of disease
Scleromyxedema is diagnosed by a suitable specialist. During the anamnesis, he discusses the patient’s medical history as well as possible chronic diseases and similar complaints in the close relatives. The person suffering from scleromyxedema describes the symptoms and the circumstances of the initial manifestation.
Finally, the treating specialist uses various clinical examination methods to diagnose the scleromyxedema. Blood tests are usually used first. The specialist then takes tissue samples from the areas of the skin affected by scleromyxedema and orders an examination in the laboratory.
The excessive accumulations of mucins in the skin can be detected. The doctor also determines the fibrosis and broken down connective tissue fibers. When diagnosing scleromyxedema, the doctor has to carry out a comprehensive differential diagnosis, since the symptoms of the disease also occur in a similar combination in numerous other diseases.
For example, the doctor excludes hillock necrosis, systemic scleroderma, acrosclerosis and Raynaud’s symptoms. Scleromyxedema must also be differentiated from localized disseminated scleroderma, systemic nephrogenic fibrosis and adult scleroedema. Finally, pretibial myxedema, eosinophilic fasciitis, and mucinoses with other causes are considered in the differential diagnosis.
If scleromyxedema is not treated, further complications usually arise over the course of the disease. Typical of the disease are skin changes such as abscesses and papules, which 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 occur.
Since the noticeable skin changes typically occur on the face and neck, those affected often perceive this as an aesthetic blemish. Mental suffering such as social anxiety and inferiority complexes can develop, which in turn are associated with complications. In individual cases, pulmonary fibrosis is seen as a late consequence of scleromyxedema. Impairments of the esophagus, disorders of the peripheral nervous system and degenerative diseases of the joints can also occur.
Dermato neuro syndrome rarely develops as a result of the dermatosis. Complications can also occur during radiation therapy. In addition to the typical radiation hangover, which is associated with headaches, nausea and vomiting, long-term effects such as changes in the genetic makeup and degeneration of tissue can also occur. In children, growth disorders can occasionally occur.
When should you go to the doctor?
Scleromyxedema usually always requires medical treatment. It is a serious skin disease that must be examined and treated by a doctor in any case. Early diagnosis is therefore very important to prevent further spread of the disease. A doctor should be consulted for scleromyxedema if papules and pustules appear. These can appear anywhere on the body and will not go away on their own.
Furthermore, severe itching on the skin indicates scleromyxedema and must then be checked by a doctor if it does not go away on its own. In many cases, the kidneys and lungs of those affected also suffer from scleromyxedema, so that these organs can experience severe symptoms. A doctor should also be consulted in this case in order 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 there are no further complications.
Treatment & Therapy
The measures and possible successes in the treatment of scleromyxedema depend primarily on the severity of the dermatosis and the therapy of the present plasmacytoma. A causative method of treating scleromyxedema is currently not available. Symptoms may be relieved with retinoids and radiation therapy with PUVA rays.
It is not yet known exactly how scleromyxedema can be effectively prevented. The causes of the skin disease have not yet been sufficiently researched. It is therefore particularly important for patients with scleromyxedema to consult 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.
After treatment of scleromyxedema, the follow-up phase begins. How this proceeds depends on the type and severity of the skin problem. An effective prevention is not yet known. This makes it so important for patients to monitor their skin and quickly make an appointment with a doctor if there are any irregularities. A short-term reaction can prevent worse consequences of the disease.
If the complexion shows abnormalities again after a treatment, those affected can readjust their skin care products. The doctor will be happy to help you with this. By abstaining from perfumed creams and cosmetics, the risk of relapse decreases. In everyday life it is possible to consciously reduce the stress on the skin. Products that contain alcohol or aggressive substances can irritate the skin’s surface.
Instead, natural-based products recommended by the treating doctor or pharmacist are recommended. In addition to protecting the skin, psychological stability also plays a role. Skin irritation can weaken self-confidence and thus negatively affect quality of life. Participation in a self-help course is very helpful in this context and counteracts inferiority complexes. When patients come to terms with their condition, it is easy for them to deal with uncomfortable situations and remain relaxed.
You can do that yourself
The possibilities of self-help are very limited in the case of scleromyxedema. Since the cause of the disease has not yet been fully clarified, no adequate therapy methods or supportive approaches have been identified.
It is therefore important to seek the cooperation of a doctor as soon as the first irregularities in the skin appear. In addition, the creams and care products used for the skin must be agreed with the doctor treating you. In order to avoid additional stress or irritation of the skin, perfumed cosmetics should not be used. It is also important to ensure that the care products do not contain any alcohol or other aggressive ingredients. It is advisable to use cosmetic products specially made for the needs of the skin.
Psychological stability is important when dealing with the disease. Self-confidence should therefore be encouraged and dealing with the disease openly is advisable. This procedure can help to prevent unpleasant situations such as looks or questions in everyday life. Wearing the clothing 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. The composition of the manufactured clothing must therefore be checked before it is worn.