Circumscribed scleroderma or morphaea 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 Morphaea?
Circumscript scleroderma (morphea) is an inflammatory disease associated with hardening (sclerosing) 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). For meanings of persistierender ductus arteriosus, please visit polyhobbies.com.
Morphaea 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.
Causes
The cause and etiology of morphaea 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
The signs and symptoms of localized scleroderma depend on whether it is the limited, disseminated, linear or deep form of the disease. The symptoms of all localized sclerodermas are defined by circumscribed, clearly definable hardening of the skin in various places. In the beginning, signs of inflammation and swelling can be noticed on the affected skin areas.
The spot may be reddish. The early signs do not suggest that it is a form of scleroderma. After several weeks, the next symptoms follow. The skin in the same place progressively hardens. This can also be accompanied by a noticeable thickening of the skin layers.
It is questionable whether the person affected will notice the shrinking of the smaller blood vessels. He is much more likely to notice a porcelain-like appearance that the affected skin areas are now taking on. The epidermis atrophies. It no longer has its usual surface structure. It looks whitish, thin and smooth.
In addition to these symptoms of localized scleroderma, hair loss or itching can occur on the affected skin areas. These are signs that the hair follicles, the sebaceous glands or the sweat glands are also affected by localized scleroderma. The skin looks dry. If there are still inflammatory processes in the skin, a reddish-purple ring can form around the affected skin area.
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 morphaea usually only show a slight improvement.
Complications
Those affected usually suffer from various skin problems with this disease. In any case, they have a negative effect on the aesthetics of those affected, so that most patients with the symptoms feel uncomfortable and suffer from inferiority complexes or reduced self-esteem. Psychological complaints are therefore not uncommon with this disease.
The skin itself is reddened and hardened. There is also swelling on the skin and in many cases severe hair loss. Those affected can also suffer from itching, which is only made worse by scratching. In most cases, there is no self-healing, so that medical treatment of the disease is necessary in any case.
The symptoms can be alleviated with the help of various therapies. There are no complications. Creams or ointments can also be helpful and have a positive effect on the course of the disease. As a rule, life expectancy is not negatively affected. In the case of psychological complaints, patients with this disease are dependent on psychological treatment.
When should you go to the doctor?
If someone notices hardening, thickening, or discoloration of certain areas of skin, they should see a doctor as soon as possible. Circumscript scleroderma could be hiding behind this phenomenon. In contrast to the systemic form of scleroderma, this form is limited to certain areas of the skin. In this respect, it is easier to treat.
The doctor can visually diagnose the circumscript scleroderma or morphea. However, it is important to do everything possible to rule out the systemic form of scleroderma. This can also harden organs. It ultimately leads to death, which is not the case for circumscript scleroderma. This rarely affects the hands and face, but rather other areas of the body. The concomitant occurrence of Raynaud’s syndrome also suggests the systemic variant rather than circumscribed scleroderma.
So if there are hardened skin areas on the hands or face and the hands also hurt and appear to have reduced blood flow, the visit to a dermatologist should be made all the faster. If necessary, the doctor will use an ultrasound scan and tissue samples to determine what form of scleroderma it is.
Whether or not circumscript scleroderma is an autoimmune disease has not yet been clarified. It is clear, however, that the cause of the hardening of the skin is an inflammation. This local skin disease can be treated well.
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 (among others at the Inselspital in Bern), which destroy the sclerosed tissue in order to stimulate blood flow 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).
Prevention
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).
Aftercare
Follow-up care for localized scleroderma is based on the individual symptoms and the course of the disease. In the case of minor symptoms, comprehensive aftercare is not necessary. The condition can be treated with medication and alternative treatments such as light therapy. As part of the aftercare, the skin areas are checked again.
If the course is positive, the patient can then be discharged. A patient consultation is also part of the aftercare. During the anamnesis, the symptoms and treatment methods are discussed. In addition, the doctor responds to individual questions from the patient, for example regarding reimbursement of costs or additional therapeutic treatment.
Young people in particular, who sometimes suffer greatly from skin changes, often need therapeutic support. If the localized scleroderma does not subside with conventional measures, alternative therapy methods must be tested. In this case, follow-up care is postponed until the condition is cured.
In the rare cases of a chronic illness, the responsible doctor must be consulted at regular intervals so that he can carry out the necessary routine checks. Follow-up care is provided by the dermatologist or general practitioner. Children and adolescents are often treated by the pediatrician, who takes over the regular follow-up examinations after the end of the treatment.
You can do that yourself
This diagnosis is very distressing for most patients, especially when the affected skin areas are on the face and/or hands and are therefore visible to everyone. If the stress is excessive, psychotherapeutic supportive treatment is to be advocated. Visiting a self-help group can also help those affected. Patients can find information about the disease and meetings nearby.
Sport, as far as the illness allows, also ensures a better mood. Participation in a team sport and subsequent socializing is recommended, especially if the patient tends towards depression. It is not yet certain how circumscript scleroderma can develop. But regardless of whether this is due to a wrong reaction of the immune system or an inflammation in the body, a healthy lifestyle should enable the body to cope better with the disease.
In addition to the sport already mentioned, a healthy lifestyle includes a selected, light diet with lots of vitamins and roughage, but with little sugar and animal fat. A regulated daily routine with sufficient sleep and rest times as well as avoiding alcohol and nicotine are also recommended. Sports such as yoga, Pilates or meditation can counteract stress that may otherwise aggravate the disease.
