Forms of the disease
Focal scleroderma combines several variants, which differ in the shape and depth of the lesion. The plaque form is most often found in adults: these are round, compacted spots on the body. The linear form is typical for children; it runs in a stripe along the limb or along the forehead and scalp, where it received the figurative name “saber strike.” Deep variants involve subcutaneous tissue, fascia and even muscles, and the generalized form produces multiple confluent lesions. Separately, a superficial variant with white shiny spots is distinguished.
- Plaque form - round dense spots
- Linear form - a stripe on a limb, more often in children
- “Saber strike” shape on the forehead and scalp
- Deep form involving fiber and fascia
- Generalized form with multiple foci
- Surface variant with white shiny spots
Reasons
The exact reason is unknown. It is believed that with a certain predisposition, the immune system triggers inflammation in the skin, and fibroblasts respond by producing excess amounts of collagen. The triggering factor can be trauma or prolonged pressure on an area of the skin, radiation therapy, some infections, and sometimes the connection cannot be identified. Women get sick more often than men. Other autoimmune diseases - thyroid, vitiligo, rheumatic diseases - are often found in patients' families. Morphea is not transmitted from person to person and is not associated with diet or skin care.
- Autoimmune inflammation with excess collagen production
- Trauma and prolonged pressure on the skin area
- History of radiation therapy
- Hereditary predisposition to autoimmune diseases
- Female gender
- The disease is not contagious
Symptoms
The onset is usually imperceptible: a pinkish or lilac spot appears, which a person may mistake for a bruise or allergy. Gradually the center brightens, becomes waxy, smooth, shiny, the skin in this place stops folding and loses hair and sweating. A purple rim around the edge indicates active inflammation. Over time, the inflammation subsides, leaving a dense area with altered pigmentation - lighter or darker than the surrounding skin. Pain and itching are moderate. Lesions on the face and over the joints in children are dangerous, as they can impair tissue growth and mobility.
- A dense, smooth stain that does not bunch up
- Lilac or purple rim in the active phase
- Waxy shine and light hearth center
- Hair loss and lack of sweating at the site
- Change in pigmentation after inflammation subsides
- Restriction of movement in case of damage above the joint
Diagnostics
An experienced dermatologist recognizes morphea by its appearance, but a skin biopsy is often performed to confirm and assess the depth of the lesion. Ultrasound of soft tissues and MRI help to understand whether tissue, fascia and muscles are involved, especially in the linear form in a child. Blood tests show signs of inflammation, and in some patients antinuclear antibodies are detected, but their presence does not mean systemic scleroderma. That is why the doctor always asks about symptoms from the internal organs: blue fingers in the cold, shortness of breath, heartburn and difficulty swallowing. If there is a linear form on the face, you need to be examined by an ophthalmologist and a neurologist.
- Examination by a dermatologist
- Skin biopsy with histological examination
- Ultrasound of soft tissues and MRI for deep forms
- Complete blood count and inflammation indicators
- Antinuclear antibodies
- Examination by a rheumatologist, if there is a form on the face - by an ophthalmologist and a neurologist
Treatment
Treatment is most effective in the active phase, while inflammation persists and a purple rim is visible: the process can be stopped, but an already formed dense scar area is much more difficult to change. For limited superficial lesions, external hormonal and other anti-inflammatory drugs are used, sometimes injecting the drug directly into the lesion. Phototherapy has a good effect. For linear, deep and generalized forms, especially in children, systemic therapy is prescribed, selected by a dermatologist together with a rheumatologist. Additionally, skin moisturizing, physical therapy and massage are important to maintain joint mobility.
- External anti-inflammatory agents for limited lesions
- Injecting drugs directly into the lesion
- Phototherapy
- Systemic therapy for linear and deep forms as prescribed by a doctor
- Therapeutic exercise and massage for lesions above the joints
- Constant skin hydration and monitoring over time