Diagnosing Pediatric Localized Scleroderma

Skin changes often look like lines or patches. They can appear on the skin’s surface, deep inside the skin, or even the layer between fat and muscles. Deep changes and can look like a divot, groove, or dimpling on the skin.

Active, inflamed skin may look pink, red, or purple. Your child may have a rim of colors in a circle around their skin lesions. These changed skin areas can feel firm, thick, or hard to move or pinch. They can look white, yellow, or like porcelain.

If skin is damages, you may see brown or white pigment changes. Skin may look thinner: it’s easy to see their veins. Your child may lose fat in these areas, so they look depressed, while other areas are thick or hard to move.

Muscles, joints, and limbs may be affected. These usually happen under or near skin lesions, especially linear lesions. Children may have muscle, joint, or limb symptoms far from lesions too.

Muscles: Your child’s muscles may look smaller on the affected side. Muscles may be weak, but it’s less common.

Joints: Joints may swell near skin lesions or deeper lesions. They may have joint tightness, pain with movement, limited range of movement, or weakness (less grip strength if their wrist or hand is affected).

Limbs: An arm, leg, or section of a limb may look shorter or narrower. This can affect hands, feet, or fingers and toes too.

Your child may have different feelings around lesions, like “pins and needles,” odd sensations, or pain. That’s because their nerves are affected. They may have these feelings in their limbs or face. Muscle twitches or spasms sometimes happen too.

Skin changes on the face or scalp may cause other symptoms, like dry eyes or vision problems. They may have headaches or, in rare cases, seizures.

Children may have patchy hair loss on the scalp. Hair may fall out in a circle or line. They may have an area that looked “dented” on their scalp too. Some children have patches of hair loss in the eyebrows, eyelashes, or mustache.

Teeth can become overcrowded and out of line. Adult teeth may have root problems. Their temporomandibular joint (TMJ) or jaw may hurt or feel stiff.

Breasts and buttocks may change on one side of the body. These areas may look uneven. That’s because deeper tissues may not grow normally or may atrophy (waste away).

Children may have acid reflux, heartburn, trouble swallowing, nausea, or belly pain. These symptoms may be caused by scleroderma changes in their esophagus, but not always. Reflux, heartburn, and stomach upset are common health problems.

It’s rare for localized scleroderma to affect the heart, lung, or kidneys. Recent studies show that a very small number of children have mild heart or lung symptoms. Usually, these don’t cause any problems and don’t get worse over time.

Read about additional tests for pediatric localized scleroderma.

Diagnosis of scleroderma may take a year or longer because many doctors don’t know much about this condition. Localized scleroderma may look like signs of infection or past injuries. Early on, people may not have any pain, stiffness, or other problems associated with the disease. Because there’s no diagnostic test right now, doctors may unkowingly misdiagnose the cause of skin changes.

Diagnosis is based on how the skin looks and feels, or joint or muscle changes, during a physical exam. If your doctor is uncertain, they should confirm diagnosis with a skin biopsy. A biopsy can show changes deep inside tissue in the skin, including inflammation and fibrosis typically seen in localized scleroderma (LS) or systemic sclerosis (SSc). They can also rule out other inflammatory skin diseases with a biopsy.

Once a diagnosis is confirmed, it’s important to begin treatment early in both children and teens with active disease. Prompt treatment can help reduce the risk for severe damage. It can also make it more likely that a child’s disease will go into remission. That’s when signs and symptoms of active skin inflammation go away altogether.