Circumscribed scleroderma or morphea is an inflammation-related skin disease that can probably be traced back to a malfunctioning immune system and which usually affects more women than men. Since the cause of the disease has not yet been clarified, localized scleroderma can only be treated symptomatically.
What is morphea?
Circumscript scleroderma (morphea) is an inflammatory disease associated with hardening (sclerotherapy) of the affected skin areas. In general, a distinction is made between the limited variant, which mainly affects the upper body, the disseminated variant, which manifests itself mainly on the upper body and in the lumbar and/or thigh area, as well as the linear variant with band-shaped skin changes on the extremities and the deep variant involving the subcutis (subcutaneous tissue).) and the fascia (Morphea profunda). See fun-wiki for What is craniostenosis used for.
Morphea initially manifests as inflammation, often causing a reddish-purple discoloration of the skin. As the disease progresses, the cells of the connective tissue increasingly form collagen fibers, as a result of which the affected areas thicken and harden, while the number of smaller blood vessels decreases.
As a rule, there is then atrophy (tissue loss) and a porcelain-like appearance of the skin with whitish discoloration and a loss of the characteristic surface relief of the epidermis. In many cases, the atrophy also causes a reduced number of hair follicles as well as sebaceous and sweat glands. The skin areas affected by localized scleroderma become dry, may be tight and/or itchy.
The cause and etiology of morphea have not yet been conclusively clarified. It is assumed that the disease is caused by a dysregulation of the immune system (autoimmune disease).
As a result of this dysregulation, the human immune system attacks the body’s own structures, presumably damaging the small blood vessels in the dermis. The cells of the affected skin areas synthesize pro-inflammatory messenger substances and growth factors that stimulate the connective tissue cells to produce more collagen fibers.
As a result, the normal balance between the build-up and breakdown of these fibers is impaired, since the increased formation of collagen fibers is offset by reduced breakdown. The connective tissue fibers accumulate in the skin areas affected by the localized scleroderma and cause hardening (sclerosing) and a loss of flexibility.
Symptoms, Ailments & Signs
Morphea can go unnoticed for weeks. The disease initially manifests itself as small spots or swellings on the skin. In the further course, these spots harden and cause an unpleasant itching. The skin on the affected area becomes dry and cracked.
Those affected often describe the complexion as whitish and porcelain-like, with the skin tissue eventually atrophying. Before that, a purple ring usually forms around the inflamed area. This can be accompanied by a feeling of tension on the skin.
In addition, the glands dry out and the hair falls out. The affected areas are usually very swollen and painful when touched. Scars and sensory disturbances remain in the long term. Physical symptoms usually do not occur with morphea. If an area of skin becomes inflamed, however, it can lead to a serious infection and, as a result, fever and other general symptoms.
Smaller 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 will go away on its own within a few days to weeks.
Diagnosis & History
Circumscribed scleroderma can usually be diagnosed on the basis of the clinical symptoms. The diagnosis is confirmed by means of a biopsy of the affected skin areas with subsequent histological examination. At the same time, this serves to differentiate it from systemic scleroderma, in which the connective tissue of the internal organs and, in many cases, of the face and hands is also affected.
Raynaud’s syndrome also points to systemic scleroderma and is considered an exclusion criterion for morphea. The thickness of the affected skin areas can also be determined as part of a sonography (ultrasound). Circumscribed scleroderma can stagnate within 3 to 5 years, so that no new foci develop. However, the areas affected by the morphea usually show only a slight tendency to improve.
In the case of morphea, patients suffer from severe and, above all, unpleasant discomfort that occurs on the skin. The skin appears tense and hardened. Itching and severe redness are not uncommon. The affected regions are often swollen, which makes those affected feel uncomfortable and ashamed of the symptoms.
Morphea can also lead to psychological limitations or inferiority complexes. Furthermore, children in particular can suffer from bullying or teasing due to the symptoms of the disease. Without treatment, the disease will also continue to lead to hair loss and dry skin. Self-healing does not occur in most cases.
The symptoms and discomfort can be relieved with the help of various therapies or creams. However, it cannot be predicted whether the course of the disease will always be positive. If the joints become stiff, physiotherapy measures are necessary. Usually there are no further complications. The patient’s life expectancy is also not reduced by this disease.
When should you go to the doctor?
If localized scleroderma is suspected, a doctor should always be consulted. People who notice changes in their hands, face or back that indicate such a disease should speak to their family doctor immediately . This applies in particular to extensive skin damage and sensory disorders in the area of the fingers. If your fingers turn white and hurt when it’s cold, or if you even experience paroxysms of pain, you should go to the nearest clinic or contact the emergency medical service.
Morphea mainly affects older people who have already suffered from a skin disease. Morphea is best treated by a family doctor or an internal medicine specialist. Other contacts are the dermatologist, rheumatologist, orthopedist or a sports doctor, depending on the cause and severity of the symptoms. If children are suspected of having localized scleroderma, they should first be taken to the responsible pediatrician. Further treatment is usually carried out in a specialist clinic for skin diseases.
Treatment & Therapy
Due to the unclear etiology of localized scleroderma, there is no causal therapy to date, and the therapeutic measures, which depend on the form and extent of the disease, are aimed at reducing the specific symptoms present.
Medication and light therapy have proven effective in alleviating symptoms. Anti-inflammatory agents such as glucocorticoids in the form of creams or ointments are used to treat the inflammatory processes. In addition, moisturizing ointments, lotions or creams are recommended for basic care of the sclerosed skin areas and to prevent itching and a possible feeling of tightness.
Existing or acute inflammatory reactions can be restricted as part of phototherapy with UVA light. At the same time, UVA light stimulates the synthesis of enzymes that break down the increased collagen. In some cases, a cream containing psoralen is applied beforehand to increase the effect (PUVA therapy). While the reddish, inflammatory skin discoloration usually recedes and the whitish areas become softer, atrophy and the loss of hair follicles cannot be reversed by therapy.
Shockwave therapy measures are also being tested as part of clinical studies (amongst others at Bern’s Inselspital), which destroy the sclerosed tissue in order to stimulate blood circulation and the synthesis of new blood vessel and skin cells. If skin areas in the joint area are affected by morphea, physiotherapeutic measures may be indicated to prevent joint stiffness (contracture).
Outlook & Forecast
The prognosis depends, among other things, on the severity of the disease. Three quarters of patients without organs such as the heart, lungs or kidneys are still alive after ten years. A disease of the kidneys, on the other hand, leads to death in the majority of cases (70 percent of those affected). The course of morphea cannot usually be predicted. Doctors are also unable to formulate any clear prospects based on the symptoms. This makes general statements difficult.
The start of treatment is essential for recovery. This is the only way to improve the prognosis. Effective drugs are now available. Complications such as organ involvement are prevented by consistent treatment. Basically, morphea is more favorable in women than in men. In science, the limiting expression is assumed to have a good outlook; the diffuse form, on the other hand, carries a high risk of death.
Sometimes restrictions remain that directly affect the quality of life. These include, above all, long-term movement restrictions. The patient then has to reorganize his professional and private everyday life. He needs help. Aids are to be used.
Since the etiology of localized scleroderma has not yet been clarified, there are no preventive measures. The effects of localized scleroderma can probably be minimized by avoiding factors that negatively influence the immune system (including stress, alcohol and nicotine consumption).
In the case of morphea, direct follow-up measures are severely limited in most cases. For this reason, the person affected is primarily dependent on rapid and, above all, very early detection and treatment of this disease in order to prevent the occurrence of other complications and symptoms. It also cannot heal itself, so a doctor should be consulted at the first signs and symptoms of morphea.
Most of those affected are dependent on taking different medicines during treatment. Care should be taken to ensure the correct dosage and regular intake in order to alleviate the symptoms permanently and correctly. Regular checks and examinations are also important in order to identify and treat changes in the skin early on.
Physiotherapy or physical therapy measures may also be necessary, although the person concerned can also carry out many of the exercises at home. As a rule, morphea does not reduce the patient’s life expectancy. Further measures or options for aftercare are usually not available to those affected by this disease.
You can do that yourself
The possibilities for self-help are limited when morphea occurs. This is because causal treatment is not possible and dealing with the symptoms is therefore crucial. However, in the case of localized scleroderma, a strengthened immune system can have a positive effect. It is believed that the effects of the disease can be reduced in otherwise perfectly healthy sufferers.
Accordingly, those affected can work on making their lifestyle as healthy as possible. Lots of vitamins and minerals, avoiding alcohol and cigarettes and moderate exercise strengthen the body. In addition, with an optimal diet, the healing times after any (cosmetic) operations can be shortened. Sugar and arachidonic acid are of particular importance here, since the latter intensifies inflammatory processes and the former promotes the absorption of the acid. However, both should be taken into account when eating in order to improve the course of the disease.
Furthermore, joints that are particularly affected (the skin changes form on them) should be used heavily. Otherwise there is a risk of the affected joint being restricted due to the tissue changes. With regard to the aesthetic restrictions from which those affected suffer, various measures to compensate can be identified. In addition to self-help groups, an adapted style of clothing or cosmetic products can also be used, for example.