TL;DR:
- Limited Scleroderma (Morphea): A rare autoimmune disorder causing hardening and thickening of the skin, typically sparing internal organs.
- Causes: Autoimmune malfunction, genetic predisposition, infections (e.g., Borrelia burgdorferi), mechanical injuries, chemical exposure, and hormonal changes.
- Symptoms: Oval/round skin patches (white, pink, or purple) that harden over time, reduced skin elasticity, atrophy of tissues, joint stiffness, and rare cases involving face/scalp changes.
- Treatment Options:
- Topical: Corticosteroids or calcineurin inhibitors.
- Systemic: Oral corticosteroids or methotrexate for severe cases.
- Other Therapies: UVA1 phototherapy for superficial lesions and surgery for deformities.
- Contraindications: Allergies to medications, active infections, or cancer history.
- Early diagnosis and treatment are crucial to prevent disease progression.
Limited Scleroderma, also known as Morphea, is a chronic autoimmune disorder that leads to hardening and thickening of the skin. Although it is a rare condition, it can cause significant changes in the appearance and function of the skin, affecting patients' quality of life. In the following post, we explain whether this disease is present, its symptoms, causes and treatment options.
What is limited scleroderma?
Limited scleroderma causes hardening and thickening of the skin. It usually affects only the skin and subcutaneous tissues, without affecting internal organs, which distinguishes it from systemic scleroderma. The disease leads to patches on the skin, which can range from white to purple in color. Over time, they harden and become less aesthetic, leading to deformities and pain in the affected areas. The causes of scleroderma are not fully known, but there are several theories about what may trigger the disease. The main factors contributing to the development of the disease include autoimmune ones. It is believed that limited scleroderma is an autoimmune disease, meaning that the immune system begins to attack its own tissues, in this case the skin and subcutaneous tissues. This can lead to inflammation and excessive collagen production, resulting in hardening of the skin. Genetic predisposition can also play a role in the development of the condition. People who have a family history of autoimmune diseases may be at higher risk for scleroderma. In addition, some bacterial or viral infections trigger or exacerbate the symptoms of scleroderma limited. For example, infection with Borrelia burgdorferi, the bacterium responsible for Lyme disease, is one potential risk factor. Mechanical injuries, such as cuts, burns or other skin damage, can sometimes trigger a local inflammatory reaction that leads to the development of scleroderma at the site of injury. Also, exposure to certain chemicals or drugs can increase the risk of developing scleroderma. Although there is no conclusive evidence, some studies suggest a link between exposure to pesticides and the occurrence of this condition. Changes in hormone levels, especially in women, may also influence the development of the disease, although the exact mechanism is not well understood.
What are the symptoms of limited scleroderma?
Symptoms of limited scleroderma can vary depending on the form of the disease and its severity. Most commonly, they involve the skin and subcutaneous tissue. The initial symptom is flat, oval or round patches on the skin, which can range in color from white to pink to purple. These spots become harder and thicker over time. The affected areas of skin become harder, stiffer and less elastic. However, the skin in these areas may be smooth and shiny. In addition, it gradually changes its color, becoming lighter or darker compared to the surrounding healthy skin. In some cases, especially with deeper lesions, there may be distortion of the subcutaneous tissues. This can lead to atrophy of muscle or fatty tissue in the affected areas. These in turn can be painful, especially if the disease involves deeper layers of skin. In more severe cases, when skin lesions occur around joints, they can cause limited mobility or joint stiffness. In rare situations, limited sclerosis also affects the scalp and face, leading to baldness in these areas or changes in facial features.
When is treatment for limited scleroderma indicated and when is it contraindicated?
Indications for treatment depend on the severity of the disease, its location, symptoms and impact on the patient's quality of life. They primarily include active skin lesions when they are visible, such as newly formed patches, worsening sclerosis. If the disease involves deeper layers of skin or subcutaneous tissue, treatment is also indicated, as it can lead to deformation or atrophy of the tissue. If the skin lesions cause reduced joint mobility, leading to contractures or permanent deformities, the doctor may also decide to institute therapy. Pain or itching caused by emerging lesions is also an indication for treatment. However, it is worth remembering that there are certain contraindications that should be taken into account before starting therapy. These contraindications may be related to specific treatment methods, individual characteristics of the Patient or coexisting medical conditions. In patients with hypersensitivity or allergy to the components of the drugs used, such as corticosteroids or immunosuppressants, the introduction of therapy with their help is discouraged. Then alternative substances that do not cause allergic reactions are used. Active bacterial, viral or fungal infections can also be a contraindication to immunosuppression, which can weaken the immune system. In addition, in patients with active tumors or a history of cancer treatment, the use of certain drugs may be risky.
How to treat limited scleroderma?
Treatment of limited scleroderma depends on many factors – such as the severity of the disease. Your doctor may recommend topical therapy with corticosteroids – anti-inflammatory drugs that are applied directly to skin lesions. They help reduce inflammation and halt the progression of sclerosis. An alternative to these substances are calcineurin inhibitors, which are used when the lesions are on delicate areas, such as the face.
Systemic treatment is included for extensive or rapidly progressive lesions that require intensive treatment. Oral corticosteroids can help control inflammation in the deeper layers of the skin. In cases that are severe, extensive or resistant to other treatments, methotrexate is used. In patients with superficial skin lesions, UVA1 phototherapy can be included to soften hardened skin and reduce inflammation. In advanced cases, when skin lesions lead to deformities or functional limitations, surgical intervention may be required. Removal of the deformed tissue or skin grafts are options for reducing the problem in such cases. Limited Scleroderma is a condition that requires early diagnosis and appropriate treatment to prevent the progression of the disease and reduce its impact on the patient's quality of life. So if you notice worrying skin changes in yourself, don't hesitate to consult your doctor. At the OT.CO Clinic, our experienced dermatologists are ready to help you fight this disease.
Conclusion
Limited scleroderma is a condition that hardens skin and harms daily life. Symptoms like skin patches, stiffness, and discoloration worsen without care. The causes, though not fully understood, involve autoimmune responses and, possibly, external triggers. Treatments, such as corticosteroids, phototherapy, or even surgery, help manage the disease and its effects. Early diagnosis and the right plan prevent serious complications. If skin changes concern you, act promptly. Managing this condition begins with awareness, so take steps toward better health today.
