Click to jump to a section:
- About Localized Scleroderma
- How Juvenile Scleroderma Is Treated
- Dealing with Peer Pressure
- How to Cope at Home
If your child has been diagnosed with a serious illness, such as scleroderma, most likely you experienced a moment of panic. You may have heard little else that your child’s doctor said after presenting you with this news. You may have rushed home and searched the Internet, your fears reinforced by the information found, and your sense of panic multiplied many times over.
It’s important to know that there are several different diseases called “scleroderma” by some doctors. Certain forms of scleroderma are quite serious, but others are much less serious. Knowledgeable doctors can treat all forms of the disease.
The first thing you must understand is which form of scleroderma your child has. Doctors divide childhood scleroderma into two forms with very different features: localized and systemic. Localized scleroderma is the most common form seen in children. This form of the disease can damage the skin, muscle, bones and joints. It is unlikely to cause damage, however, to your child’s internal organ systems. Systemic scleroderma, often times, causes internal organ damage and may take a more severe course.
If you don’t know whether your child has localized or systemic scleroderma, you should consult with your doctor. Once a doctor or specialist evaluates your child, you should have a definite answer about the form of the disease, in which your child has.
Localized scleroderma can be divided into the following groups:
- Linear scleroderma
- Scleroderma en coup de sabre.
Your child may have more than one type of the disease.
Morphea is the most common form of localized scleroderma. Your child may have irregular patches of skin on the arms, leg or body. These patches may start out small and pink. You may not have noticed this change in skin until it reached the size of a penny or larger. Many different skin rashes and infections could cause similar-looking lesions. A variety of creams or ointments could have been tried until your child was referred to specialist.
Later, the patch of skin may become pale, dry and hard. This change could lead to a scleroderma diagnosis. Sometimes, a diagnosis can be made by the visual appearance of lesions on the skin. Other times, it’s necessary to do a skin biopsy, a procedure where a doctor will take a small piece of skin tissue to examine under a microscope. Once a proper diagnosis has been made, a treatment plan can be started.
Children with morphea have an excellent outlook for the future. In the beginning, these patches of skin may continue to enlarge or increase in number. But, they will soften and darken in the future. While the period where your child has active lesions of morphea is worrisome and unpleasant, most often, there are no serious long-term consequences. Although parents would like the doctor to say that there will be no new lesions, or that the lesions will look better by a specific date, this is not possible. Morphea evolves over years, but rarely worsens once it clearly begins to improve.
Linear scleroderma is another form of localized scleroderma. It refers to a type of skin involvement that appears to spread out along lines. Instead of an almost-round area or patch of skin involved, you may notice a streak skin involved in your child. Many times, this appears on an arm or leg, and then extends on to the hands or feet. Generally, only one arm or one leg is involved.
Under the microscope, the lesions of linear scleroderma look just like those of morphea. Small areas of linear scleroderma rarely cause trouble and are treated in the same way as morphea. However, larger areas of linear scleroderma that extend over an arm or leg, or cross a joint (such as an elbow, wrist, shoulder, knee, ankle or finger), may cause permanent damage. As the skin becomes tight and the lesions harden, they can limit the ability of the joint to move. Your child may have trouble using his/her arm or leg. Also, when large areas of an arm or leg are affected in young children, the limb may not grow properly. As a result, children with linear scleroderma involving large areas or crossing joints most often are treated more aggressively than children with morphea.
The outlook for children with linear scleroderma who receive appropriate treatment is good. However, there may be long-term damage to involved fingers or toes, or permanent changes in the size of an arm or leg as result of untreated or unresponsive linear scleroderma. The skin lesions themselves typically soften and turn light brown over a period of years, as seen in patches of morphea.
Linear scleroderma en coup de sabre is the term generally applied when children have linear scleroderma on the head or face. Originally, this term applied to children with a deep furrow along the scalp with tight, hard skin that often extended to the forehead.
Children with linear scleroderma en coup de sabre are a very diverse group. Some children appear to have the classically described disease with lesions only on the scalp and forehead, while other children may have lesions only on the chin or lip. There is another group of children who are termed as having Parry Romberg syndrome. Children with this condition have similar skin lesions, but may have involvement of the whole side of the face and even involvement of the tongue. Obvious cases of en coup de sabre and Parry Romberg syndrome differ significantly, but many children present with crossover manifestations, which can make it difficult to determine with certainty, which form of scleroderma the child has.
The long-term outcome for children with linear scleroderma en coup de sabre is mixed. If the lesions are confined to the scalp and forehead, they often evolve similarly to linear scleroderma and the effect is primarily cosmetic. The same is true for isolated areas of involvement on the face. Parry Romberg syndrome, in which one side of the face is involved, can present additional serious difficulties because the bones may not grow properly
Appropriate treatment for children with localized scleroderma depends on the area of skin involved. Small areas on the body may not need any therapy while lesions on the face or over the joints often are treated aggressively. For children with mild morphea, topical treatment with calcipotriene cream or ointment during the active phase is often sufficient. Linear scleroderma often responds well to methotrexate, and physicians may use it for children with widespread or otherwise disturbing morphea.
Linear scleroderma that does not cross a joint line and is not of sufficient extent to suggest it will cause deformity, probably does not require treatment. However, when large areas of an extremity are involved, or the disease is crossing a joint line, there is a significant risk of permanent damage. In these cases, most specialists will recommend therapy with methotrexate, which is often associated with a slow but steady softening of the involved skin. Like all medicines, methotrexate is not without possible side effects and careful consideration should be given to both the expected risks and benefits. The use of methotrexate for children with linear scleroderma en coup de sabre remains controversial. Discuss the benefits and risks of the medication with your child’s doctor. Proper therapy for children with Parry Romberg syndrome is unknown.
Although there are no scientific studies, many families have found the application of commercially available cocoa butter preparations useful. Many doctors suggest you avoid food additives or dietary supplements, as they can be potentially harmful.
How your child interacts with other children is one of the most important subjects that doctors frequently overlook. Children with chronic disease who either have visible effects, such as morphea or linear scleroderma, need to know what to do when “somebody says something.”
For some children, the psychosocial impact of scleroderma may be the most severe aspect of the disease. What kids and families say when asked about “those spots” on his/her arm or leg is not as important as the effect that having to formulate an answer and respond to comments can be. Too many people fall into the trap of either trying to give a complete explanation or being defensive. Neither is appropriate. The key is to develop a practiced answer that minimizes the stress for the patient and their family.
Another area to consider is how you and your child cope with scleroderma at home. It can be far more complicated and important than dealing with others. Your child may ask you, “What did I do wrong to get this disease?”
It’s important to have open discussions with your child, and provide him/her with reassurance. However, sometimes professional help is necessary. No one should be ashamed to seek the help they need to deal with the stress of chronic illness in themselves or a family member. If emotional needs from the stress of living with a chronic illness are left unmet, this may lead to your child not following treatment plans, or it could event worsen the disease outcome.
Juvenile scleroderma can be unsettling for you and your child. But, an experienced doctor and other health care professionals can treat it properly. If you need help finding a physician, call the Scleroderma Foundation at (800) 722-HOPE (4673).
Please note that this brochure is provided for educational purposes only. It is not intended to substitute for informed medical advice.
The Scleroderma Foundation thanks Thomas J. A. Lehman, M.D., Hospital for Special Surgery, for his assistance in the preparing this article.