Circumscribed Scleroderma: Causes, Symptoms & Treatment

Circumscribed scleroderma, or morphaea, is the name given to an inflammation-related skin disease that is probably attributable to a malfunctioning 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 morphaea?

Circumscribed scleroderma (morphaea) 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, morphaea 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 morphaea 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

The complaints and symptoms of circumskriptenous scleroderma depend on whether it is the limited, disseminated, linear, or deep form of the disease. Symptoms of all circumscribed scleroderma are defined by circumscribed, clearly demarcated indurations of the skin at various sites. Initially, signs of inflammation and swelling may be noticed at the affected skin sites. The area 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 area becomes increasingly hardened. This may also be accompanied by a noticeable thickening of the skin layers. Whether the affected person notices the shrinking of the smaller blood vessels is questionable. It is much more likely that they will notice a porcelain-like appearance that the affected skin areas now take on. The epidermis atrophies. It no longer has its usual surface structure. It appears whitish, thin and smooth. In addition to these symptoms of circumscritic scleroderma, hair loss or itching may occur in the affected skin areas. These are signs that the hair follicles, sebaceous glands or sweat glands are also affected by circumskripten scleroderma. The skin appears dry. If inflammatory processes are still present in the skin, a reddish-purple ring may form around the affected area of skin.

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 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.Likewise, Raynaud’s syndrome indicates systemic scleroderma and is considered an exclusion criterion for morphaea. 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 morphaea usually show only a slight tendency to improve.

Complications

In this disease, affected individuals usually suffer from various skin complaints. In any case, they have a negative effect on the aesthetics of those affected, so that most patients feel uncomfortable with the complaints, suffering from inferiority complexes or reduced self-esteem. Psychological complaints therefore also occur not infrequently with this disease. The skin itself is reddened and hardened. Furthermore, there is also swelling on the skin and in many cases severe hair loss. Those affected may also suffer from itching, which is only further intensified by scratching. In most cases, there is no self-healing, so that medical treatment of the disease is necessary in any case. With the help of various therapies, the symptoms can be alleviated. Complications do not occur. Furthermore, creams or ointments can 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 one go to the doctor?

If someone notices hardening, thickening or discoloration of certain areas of the skin, he should see a doctor as soon as possible. Circumscribed scleroderma may be hiding behind this phenomenon. Unlike the systemic form of scleroderma, this form is limited to certain areas of the skin. In this respect, it is more treatable. The physician can diagnose circumscripte scleroderma or morphea by visual inspection. However, it is important to do everything possible to exclude the systemic form of scleroderma. This is because it can also harden organs. It ultimately leads to death, which is not the case for circumscritic scleroderma. It also 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 circumscripte scleroderma. Therefore, if hardened areas of skin are present on the hands or face and, in addition, the hands are painful and appear to be poorly perfused, a visit to a dermatologist should be made all the more quickly. If necessary, the doctor will use an ultrasound examination and tissue sampling to determine which form of scleroderma is involved. Whether circumscripte scleroderma is an autoimmune disease or not has not yet been clarified. What is clear, however, is that the cause of the skin hardening is inflammation. This local skin disease can be treated well.

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 clinical studies, shock wave therapy measures are also being tested (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 region are affected by morphaea, physiotherapeutic measures may be indicated to prevent joint stiffness (contracture).

Prevention

Because the etiology of circumskriptenous 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, nicotine use).

Follow-up

Follow-up care for circumscritic scleroderma is based on the individual symptom picture and the course of the disease. Mild symptoms do not require extensive follow-up. The disease can be treated with medication and alternative treatment methods such as light therapy. As part of the aftercare, the skin sites are checked again. If the course is positive, the patient can then be discharged. Part of the aftercare is also a patient interview. During the anamnesis, the symptoms and treatment methods are discussed. In addition, the doctor addresses the patient’s individual queries, for example regarding cost coverage or accompanying therapeutic treatment. Adolescents in particular, who sometimes suffer severely from skin changes, often require therapeutic support. Insofar as circumscripital scleroderma does not subside through conventional measures, alternative therapeutic methods must be tested. In this case, follow-up care is postponed until the condition is cured. In the rare cases of chronic disease, the responsible physician must be visited at regular intervals so that he can perform 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 will provide regular follow-up after the end of treatment.

What you can do yourself

This diagnosis is very distressing for most patients, especially if the affected areas of skin are on the face and/or hands and thus visible to all. If the stress becomes excessive, psychotherapeutic adjunctive treatment is advocated. Attending a self-help group can also help those affected. Patients can find information on the disease and on meetings in the vicinity, for example, at the self-help portal www.sklerodermie-sh.de. Sport, as far as the illness permits, also provides for a better mood. Particularly if patients are prone to depression, it is advisable to participate in a team sport and the subsequent social gatherings. It is not yet certain how circumskripten scleroderma can develop. However, regardless of whether it is a wrong reaction of the immune system or an inflammation in the body, an emphasized healthy lifestyle should allow the body to better cope with the disease. A healthy lifestyle includes, in addition to the aforementioned exercise, a selected, light diet with plenty of vitamins and fiber, but with little sugar and animal fat. A regular daily routine with sufficient sleep and rest periods as well as abstaining from alcohol and nicotine are also recommended. Sports such as yoga, Pilates or even meditation can counteract stress, which may otherwise aggravate the condition.