Summary about Disease
Localized scleroderma is a chronic autoimmune disease that affects the skin and underlying tissues. It causes hardening and tightening of the skin in localized areas, but unlike systemic sclerosis, it typically does not affect internal organs. There are two main types: morphea, which presents as patches of discolored, hardened skin, and linear scleroderma, which appears as lines or bands of thickened skin, often on the limbs or forehead.
Symptoms
Symptoms vary depending on the type of localized scleroderma:
Morphea:
Oval-shaped patches of thickened, discolored skin. These patches may be white, reddish, or purplish.
The patches can be itchy or painful.
Patches can appear anywhere on the body, but are most common on the trunk, arms, and legs.
Linear Scleroderma:
Lines or bands of thickened, hardened skin.
These lines often occur on the arms, legs, or forehead (en coup de sabre).
Linear scleroderma can sometimes affect underlying muscles and bones, leading to growth problems in children.
Joint pain.
Causes
The exact cause of localized scleroderma is unknown, but it is believed to be an autoimmune disorder. This means that the body's immune system mistakenly attacks its own tissues. Genetic predisposition, environmental factors, and infections may play a role in triggering the disease.
Medicine Used
Topical Corticosteroids: To reduce inflammation and slow the progression of skin changes.
Topical Calcipotriene: A vitamin D analog that can help soften the skin.
Phototherapy (UV light therapy): UVA or UVB light can help to reduce inflammation and improve skin texture.
Methotrexate: An immunosuppressant drug that can slow down the disease progression, especially in linear scleroderma.
Corticosteroids (oral or injected): Used to reduce inflammation.
Other Immunosuppressants: Mycophenolate mofetil, azathioprine, are sometimes used.
Physical therapy: Helps to maintain mobility and prevent contractures, especially in linear scleroderma.
Is Communicable
No, localized scleroderma is not contagious. It cannot be spread from person to person through any form of contact.
Precautions
While there are no specific precautions to prevent localized scleroderma, the following may be helpful:
Protect skin: Keep the skin moisturized and protected from sun exposure.
Manage stress: Since stress can sometimes exacerbate autoimmune conditions, stress management techniques may be beneficial.
Physical Therapy: Maintain range of motion and mobility.
Early Diagnosis: Early diagnosis and treatment can help to slow the progression of the disease and prevent complications.
How long does an outbreak last?
The duration of an outbreak is variable. Localized scleroderma can be active for several years, followed by a period of inactivity. The active phase, where new lesions may appear or existing ones worsen, can last from months to years. Eventually, the disease often becomes inactive (burns out), but the skin changes may persist.
How is it diagnosed?
Diagnosis typically involves:
Physical Examination: A doctor will examine the skin for characteristic signs of localized scleroderma.
Skin Biopsy: A small sample of skin is removed and examined under a microscope to confirm the diagnosis.
Blood Tests: Blood tests may be done to rule out other conditions and to look for signs of inflammation.
Imaging Studies: In cases of linear scleroderma, MRI or ultrasound may be used to assess involvement of underlying tissues.
Timeline of Symptoms
The timeline can vary greatly from person to person:
Initial Stage: The skin may appear slightly discolored, red, or inflamed.
Hardening Stage: Over time, the affected skin becomes thickened, hardened, and tight. This stage can last for months to years.
Quiescent Stage: The disease may become inactive, and the skin may soften slightly. However, the discoloration and skin changes may persist.
Complications: In linear scleroderma, joint contractures or growth problems may develop over time, especially in children.
Important Considerations
Localized scleroderma is not the same as systemic sclerosis. It typically does not affect internal organs.
Early diagnosis and treatment are important to slow the progression of the disease and prevent complications.
Regular follow-up with a dermatologist or rheumatologist is necessary to monitor the condition and adjust treatment as needed.
Physical therapy and occupational therapy can help to maintain mobility and function.
Support groups and online communities can provide valuable information and support for people with localized scleroderma.
Children with linear scleroderma, particularly affecting the face or limbs, require careful monitoring for growth disturbances and may benefit from aggressive early treatment to minimize long-term complications.