Understanding the Different Types of Scleroderma

At Pacific Rheumatology Medical Center in Laguna Hills and Tustin, California, board-certified rheumatologist Dr. Behnam Khaleghi provides highly attentive, advanced care for people living with autoimmune diseases such as scleroderma. 

This chronic autoimmune disease is complex and can vary dramatically from one person to the next, often leading to confusion and misunderstanding about its different types. 

All forms of scleroderma share the characteristic of excessive collagen production. This protein helps keep skin firm and strong, but having too much of it can cause both thickening and hardening of the skin. In some cases, it affects organs such as the lungs, heart, and digestive system. 

Because the disease can vary widely, understanding its primary types can be key to finding the right treatment plan.

Localized scleroderma

Localized scleroderma is the most common type, affecting around 66% of people with the condition. It’s most common in children, but it can also affect adults. Women are far more likely to develop localized scleroderma than men.

This type of scleroderma affects the skin and tissues underneath, but not internal organs. The two forms of localized scleroderma are morphea and linear scleroderma. 

Morphea

Morphea causes patches of hard, shiny, or discolored skin. They’re often round or oval-shaped and may vary in color, but are usually pale. The most common sites for morphea are the trunk (the chest, abdomen, and back) and the limbs. 

With morphea, the skin may feel unusually hard and tight, and it won’t typically stretch as easily as the skin nearby. It may be uncomfortable, though not necessarily painful.

Linear scleroderma

Linear scleroderma causes thick streaks or lines of hardened skin that often appear on the arms, legs, or face. 

It often looks like a scar running down an arm, leg, or face. When it occurs on either the face or scalp, it’s often called en coup de sabre (French for “strike of the sword”) due to its slash-like appearance.

Linear scleroderma can reach into the deeper layers of tissue, sometimes affecting the muscles, bones, and joints. However, it doesn’t affect the internal organs. 

If not treated, linear scleroderma may significantly restrict movement. En coup de sabre may occur alongside seizures in rare cases.

Systemic scleroderma

Systemic scleroderma, also known as systemic sclerosis, affects both the skin and internal organs. It’s about five times more common in women. It is divided into limited cutaneous and diffuse cutaneous forms. 

Limited cutaneous systemic sclerosis 

Limited cutaneous systemic sclerosis develops slowly and mainly affects the skin on the hands, forearms, face, and lower legs. It can also cause lung or digestive symptoms over time. 

Diffuse cutaneous systemic sclerosis 

Diffuse cutaneous systemic sclerosis progresses faster and involves larger areas of skin. It can affect major organs like the heart, lungs, and kidneys, so close medical supervision is important.

Sine scleroderma

Sine scleroderma is a rare form of systemic scleroderma that affects internal organs but not the skin. It accounts for about 8% of systemic sclerosis cases.

Because it lacks the visible skin thickening of other forms, it can be more difficult to detect. Diagnosis often depends on lab testing and imaging to identify internal changes caused by the disease.

Overlap syndromes

Overlap syndromes happen when a person has scleroderma along with another autoimmune condition, such as lupus or rheumatoid arthritis. You need specialized care from a rheumatic disease expert like Dr. Khaleghi to manage multiple immune system problems at once.

Scleroderma solutions

Although experts continue to search for a cure for scleroderma, it’s important to know that early diagnosis and expert disease management can dramatically improve your quality of life. 

Dr. Khaleghi takes a nonsurgical, comprehensive approach tailored to your type of scleroderma and attuned to your unique needs. Treatment options may include lifestyle changes, biologic disease-modifying anti-rheumatic drugs (DMARDs) via IV therapy, and advanced regenerative medicine options. 

Our goal is to help delay or stop disease progression and help you reclaim your active, vibrant life.

If you or a loved one is living with scleroderma, contact Pacific Rheumatology Medical Center in Laguna Hills at 707-607-8912 or in Tustin at 714-266-1458 to schedule a consultation with our caring specialists.