Circumscribed scleroderma or morphea is the name given to an inflammation-related skin disease that is probably attributable to a dysregulated immune system and which generally affects more women than men. Because the cause of the disease has not yet been determined, circumscritic scleroderma can only be treated symptomatically.
What is morphea?
Circumscribed scleroderma (morphea) is an inflammatory disease associated with hardening (sclerosis) of affected areas of the skin. In general, a distinction is made between the limited variant, which mainly affects the upper body, the disseminated variant, which manifests mainly on the upper body and in the lumbar and/or thigh areas, and the linear form with band-like skin changes on the extremities and the deep form with involvement of the subcutis (lower skin) and fascia (morphea profunda). Initially, morphea is manifested by inflammation, which often causes a reddish-purple skin discoloration. As the disease progresses, the cells of the connective tissue produce more collagen fibers, causing the affected areas to thicken and harden, while the number of smaller blood vessels decreases. As a rule, atrophy (tissue loss) and a porcelain-like appearance of the skin with whitish discoloration and loss of the characteristic surface relief of the epidermis subsequently occur. In many cases, atrophy also causes a reduced number of hair follicles and sebaceous and sweat glands. Areas of skin affected by circumscritic scleroderma become dry, may become taut, and/or itchy.
Causes
The cause and etiology of morphea have not been conclusively determined. It is suspected that the disease is caused by a dysregulation of the immune system (autoimmune disease). As a result of this dysregulation, the human immune system is directed against the body’s own structures, which presumably damages the small blood vessels of the dermis. The cells of the affected skin areas synthesize pro-inflammatory messenger substances and growth factors, which stimulate the cells of the connective tissue to produce more collagen fibers. As a result, the normal balance of formation and degradation of these fibers is impaired, as the increased formation of collagen fibers is matched by decreased degradation. The connective tissue fibers accumulate in the areas of skin affected by circumscritic scleroderma, causing hardening (sclerosis) and loss of flexibility.
Symptoms, complaints, and signs
Morphea may go unnoticed for weeks. The condition initially manifests as small patches or swellings on the skin. As the condition progresses, these patches harden and cause uncomfortable itching. The skin becomes dry and cracked in the affected area. Those affected often describe the skin appearance as whitish and porcelain-like, with the skin tissue eventually atrophying. Prior to this, a purple ring usually forms around the inflamed area. This may be accompanied by a feeling of tightness on the skin. In addition, the glands dry out and the hair falls out. The affected areas are usually very swollen and painful to the touch. In the long term, scars and sensory disturbances remain. Physical symptoms usually do not occur with morphea. However, when a skin site becomes inflamed, a severe infection may develop, followed by fever and other general symptoms. Younger children in particular also suffer mentally from morphea. The noticeable skin changes can lead to social anxiety and inferiority complexes, especially when they occur as a result of exclusion or teasing. Morphea itself is relatively harmless and resolves on its own within a few days to weeks.
Diagnosis and course
In all cases, circumscritic scleroderma can be diagnosed on the basis of clinical symptoms. Confirmation of the diagnosis is based on a biopsy of the affected skin areas followed by histologic examination. This also serves to distinguish it from systemic scleroderma, in which the connective tissue of the internal organs and, in many cases, the face and hands are also affected. Likewise, Raynaud’s syndrome points to systemic scleroderma and is considered an exclusion criterion for morphea. Sonography (ultrasound) can also be used to determine the thickness of the affected areas of skin.Circumscribed scleroderma may stagnate within 3 to 5 years, so that no new foci develop. However, areas affected by morphea usually show only a slight tendency to improve.
Complications
In the case of morphea, patients suffer from severe and, above all, unpleasant discomfort that appears on the skin. The skin appears tense and hardened. It is not uncommon to experience itching and severe redness. The affected regions are not infrequently swollen, making the sufferers feel uncomfortable and ashamed of the discomfort. At the same time, morphea can also lead to psychological limitations or inferiority complexes. Furthermore, children in particular may suffer from bullying or teasing due to the symptoms of the disease. Without treatment, the disease further also leads to hair loss and dry skin. Self-cure does not occur in most cases. The symptoms and discomfort can be alleviated with the help of various therapies or creams. However, it cannot be predicted whether there will always be a positive course of the disease. If stiffening of the joints occurs, physiotherapy measures are necessary. As a rule, this does not lead to further complications. The patient’s life expectancy is also not reduced by this disease.
When should one go to the doctor?
In any case, a doctor should be consulted if circumstrict scleroderma is suspected. Individuals who notice changes on the hands, face, or back that are indicative of such a condition are best to speak with their primary care physician immediately. This is especially true in the case of extensive skin damage and sensory disturbances in the area of the fingers. If the fingers turn white and hurt when cold, or if there are even seizure-like pains, the nearest clinic should be visited or the emergency medical service contacted. Morphea predominantly affects older people who have had a skin disease before. Morphea is best treated by a family physician or a specialist in internal medicine. Other contacts are the dermatologist, rheumatologist, orthopedist or a sports medicine specialist, depending on the cause and severity of the symptoms. Children should first be taken to the appropriate pediatrician if circumscritic scleroderma is suspected. Further treatment is usually given in a specialized clinic for skin diseases.
Treatment and therapy
Because of the unexplained etiology of circumscritic scleroderma, no causal therapy exists to date, and therapeutic measures, which depend on the form and extent of the disease, are aimed at reducing the specific symptoms present. Medicinal and light therapy measures have proven effective in alleviating the symptoms. Anti-inflammatory agents such as glucocorticoids in the form of creams or ointments are used to treat the inflammatory processes. In addition, lipid-replenishing ointments, lotions or creams are recommended for basic care of the sclerosed skin areas and to prevent itching and a possible feeling of tension. Existing or acute inflammatory reactions can be restricted with UVA light in the course of phototherapy. At the same time, UVA light stimulates the synthesis of enzymes that break down the increased collagen. In some cases, a psoralen-containing cream is applied in advance to enhance the effect (PUVA therapy). While the reddish inflammatory skin discolorations usually disappear and the whitish areas become softer, atrophy and the loss of hair follicles cannot be reversed by the therapy. In addition, within the framework of clinical studies, shock wave therapeutic measures are being tested (e.g. at the Inselspital in Bern), by which the sclerosed tissue is destroyed in order to stimulate blood circulation and the synthesis of new blood vessel and skin cells. If skin areas in the joint region are affected by morphea, physiotherapeutic measures may be indicated to prevent joint stiffness (contracture).
Outlook and prognosis
The prognosis depends, among other things, on the severity of the disease. Patients without disease of organs such as the heart, lungs, or kidneys are three-quarters still alive after ten years. In contrast, disease of the kidneys leads to death in the majority of cases (70 percent of those affected). The course of morphea cannot usually be predicted.Doctors cannot formulate a clear outlook even on the basis of the complaints. This makes general statements difficult. It is essential for recovery to start treatment. This is the only way to improve the prognosis. In the meantime, effective drugs are available. Complications such as organ infestation do not occur with consistent treatment. In principle, morphea progresses more favorably in women than in men. In science, the limiting form is thought to have a good outlook; in contrast, the diffuse form carries a high risk of death. Sometimes, limitations remain that directly affect quality of life. These include, above all, long-term movement restrictions. The patient then has to reorganize his professional and private daily life. He is dependent on help. Auxiliary means are to be used.
Prevention
Because the etiology of circumscritic scleroderma has not yet been determined, no preventive measures exist. The effects of circumscritic scleroderma can probably be minimized by avoiding factors that negatively affect the immune system (including stress, alcohol, and nicotine use).
Follow-up
In most cases of morphea, measures of immediate follow-up are severely limited. For this reason, the affected person is primarily dependent on rapid and, above all, very early detection and treatment of this disease to also prevent the occurrence of other complications and complaints. It is also not possible for the disease to heal itself, so a doctor should be consulted at the first signs and symptoms of morphea. Most of the patients depend on taking different medicines during the treatment. Attention should be paid to the correct dosage and regular intake in order to alleviate the symptoms permanently and correctly. Furthermore, regular checks and examinations are important in order to detect and treat changes in the skin at an early stage. Measures of physiotherapy or physical therapy may also be necessary, whereby the affected person can also perform many of the exercises at home. As a rule, morphea does not reduce the patient’s life expectancy. Other measures or options for follow-up care are usually not available to the affected person with this disease.
Here’s what you can do yourself
The options for self-help are limited when morphea occurs. This is because causal treatment is not possible and, accordingly, dealing with the symptoms is crucial. However, in the case of circumscritic scleroderma, it is the case that a strengthened immune system can have a positive effect. It is believed that the effects of the disease can be reduced in otherwise completely healthy affected individuals. Accordingly, affected individuals can work on their own to make their lifestyle as healthy as possible. Plenty of vitamins and minerals, avoidance of alcohol and cigarettes, and moderate exercise strengthen the body. In addition, an optimal diet can also shorten healing times after any (cosmetic) operations. Sugar and arachidonic acid are of particular importance here, as the latter increases inflammatory processes and the former promotes the absorption of the acid. However, both should be taken into account in food intake for the benefit of a better course of the disease. Furthermore, especially affected joints (the skin changes form on them) should be used heavily. Otherwise, due to the changes in the tissue, there is also a risk of restriction of the affected joint. With regard to the aesthetic limitations suffered by those affected, various measures can be identified to compensate for them. In addition to self-help groups, for example, an adapted clothing style or cosmetic means can be applied.
