Juvenile Dermatomyositis (JDMS) What Is It?
Juvenile dermatomyositis (der-MA-toe-My-o-SY-tis)(JDMS) is a disease that causes a skin rash and weak muscles in children. JDMS is different from dermatomyositis in adults, and from polymyositis* (PAH-lee-My-o-SY-tis), a disease that usually affects adults.
The skin rash and weak muscles of JDMS are caused by inflammation in the blood vessels that lie under the skin and in the muscles. This is called vasculitis. Since blood vessels run throughout the body, JDMS can also affect other areas such as the digestive tract. Usually, the worst symptoms are in the skin and muscles.
JDMS is often called an autoimmune disease. The immune system is a group of cells which normally protects the body from infections. In autoimmune diseases, these cells are signaled or triggered to "turn on" their infection-fighting process, but then can't turn it off. The process then ends up damaging the body rather than protecting it.
One way the immune system cells fight infection is by a process called inflammation (in-flah-MAY-shun). But when the cells can't "turn off" the inflammation process, tissues are damaged. In JDMS, blood vessels in the skin and muscles are inflamed. An inflamed muscle will feel weak or sore, while inflamed skin will have a rash.
We don't know what causes JDMS and why some children get it and others do not. Once the immune system is turned on it will damage it's intended enemy, in this situation, the body itself. Many researchers now think the autoimmune reaction may be caused by the immune system's abnormal response to a virus. That is, immune system cells attack an invading virus, but once the virus is gone, the immune system does not realize that it is time to stop the fight and attacks the body's own tissue(s). We now also know that the tendency for the immune system to react in this way is, at least, partially inherited. This is not a simple genetic inheritance, there is usually only one affected person in the entire extended family, but we do think that there are genes or groups of genes which either allow for the autoimmune illness to occur or fail to protect the person from developing it.
JDMS is not contagious. Your child didn't catch it from another child, and nothing you could have done would have prevented your child from developing this disease.
There are three main symptoms of JDMS: skin
rash, weak muscles, and fever. The skin rash is usually first, and weak muscles
follow, usually very slowly and quietly rather than suddenly. Some children may
also have other symptoms such as sore or swollen joints and fever. Almost all
children with JDMS have less energy and do fewer activities or just refuse to
do certain tasks because they know they cannot do them successfully. Many
parents of children with JDMS blame themselves for not noticing weakness
earlier, they feel guilty about thinking or even worse, saying, that their
child is lazy or slow. This is very common. Many physicians fail to recognize
this illness during its early stages; it is therefore not surprising that it
goes unrecognized, often for weeks or months.
Remember: JDMS can be mild in some children and severe in others. Your child may not have all of these symptoms, and may have only minor problems with some of them. For example, some children with JDMS may only complain of inability to play on the monkey bars while others may become weak so quickly as to be unable to rise from bed or stand up from floor.
The skin rash usually occurs on the face, knuckles, elbows, knees, and ankles. In some children, the rash may cover the whole body. The rash usually appears before muscle weakness becomes apparent but it may occur after or at the same time as the muscle weakness. The rash may be made worse by sunlight.
What you may notice: Your child's eyelids
and cheeks may appear red or purplish, and the eyelids may become puffy. Red patches that look like dry skin
appear over the knuckles, elbows, and knees. The cuticles may appear red and
swollen as though the child has been biting them. Because of the puffy eyelids
and skin rash, this part of the illness can look very much like an allergy and
is often diagnosed as such. The dry rash over knees and elbows may be
misdiagnosed as eczema. Since the rash may be made worse by sunlight, children
should wear sunscreen when they are in the sun.
The body's central muscles, those that are in and near the trunk, such as the neck, shoulders, back, abdomen, and hips will slowly become very weak. The muscles used for swallowing and breathing may also become weak. This happens after the central muscles are already affected and could be dangerous and be the reason the child with JDMS is admitted to the hospital.
What you may notice: Your child is likely to have trouble with common movements such as climbing stairs, climbing into a car or a bus, riding a bicycle, or getting out of a bathtub. It may also be difficult to get up from the floor or from a low chair. Your child will probably have a hard time keeping up physically with children the same age, may fall more often than usual, and may often feel tired. Children with JDMS may also complain that their muscles are sore to touch.
In very severe cases of JDMS, even the muscles used for swallowing can be affected. Your child may have trouble swallowing chunks of food such as meat and may choke on some foods. These same muscles are used for speaking, so your child's voice may become weak, and she may sound as if she is talking through her nose or simply run out of voice. If you or your child notice any of these symptoms, contact the doctor immediately.
At the start of JDMS, some children have a fever along with the other symptoms described above.
What you may notice: Your child's temperature will rise
one or two degrees.
OTHER POSSIBLE SYMPTOMS
The following symptoms may also occur in JDMS. Some are less common, but since they can occur you should know about them.
Stiff and Swollen Joints
In JDMS, one or several joints may become stiff or inflamed (swollen). The joints feel stiff or sore because the muscles around them are inflamed, or because the joint itself is inflamed. Unlike other forms of childhood arthritis, the inflammation usually doesn't last long or cause joint damage.
What you may notice: Your child may complain of stiff
and sore joints. The joint may also feel a bit warm when you touch it, it may
be swollen and even red.
A vasculitic ulcer is a hole in the tissue that surrounds an inflamed blood vessel. In JDMS, vasculitic ulcers can occur in the skin and in the bowels (the gastrointestinal tract through which food travels from the esophagus to the rectum).
In the skin: These ulcers happen when the blood vessels under the skin are severely inflamed. They look like sores in the skin rash, and can be as small as a pencil point or larger than a silver dollar. Some ulcers are very deep, while others occur only on the surface. They can be very painful, and can last anywhere from a few days to several months. The ulcers usually go away with treatment.
In the bowels: These ulcers happen when the blood vessels in the protective lining around the bowels are inflamed. This causes a break, or hole, in the lining. Strong digestive juices can then leak through that hole and damage digestive organs. If this happens to your child, you may notice these symptoms:
· severe pain in the abdomen
· coal-black stools
· change in pattern of bowel movement
· passing blood in stools.
This happens only rarely, but if you or your child notice any of these symptoms contact the doctor immediately.
Calcinosis (kal-sih-NO-sis) is a process that causes small lumps of calcium to form under the skin or in the muscle. The lumps may feel like little rocks under the skin and can range from the size of a period on this page to the size of a flat pebble. Sometimes, the smaller lumps join together to form larger calcium lumps. When this happens, it can impair muscle movement. The lumps are not caused by too much calcium in your child's diet - they are a late result of the illness and occur in more than half of all children with JDMS. Calcium lumps are usually not present at the beginning of the illness. It is thought that calcium is more likely in children who have untreated muscle inflammation either because they were not diagnosed in time or were not treated aggressively enough to control inflammation promptly. It is possible however that no matter what is done or not done, some children with JDMS are at risk for calcinosis as part of their particular illness.
the calcium lumps are bumped a lot, they can become sore. This is most likely
to happen if they are on your child's knee, elbow, buttocks, or other pressure
joint. Small pieces of creamy white calcium may break through the skin and
drain from the hole. These draining areas will usually heal, but they can
become infected. Contact your doctor if this happens.
In most children, after a while, the calcium lumps are usually absorbed back into the body. Rarely, the calcinosis becomes extensive and significantly affects movement.
A contracture is a shortened muscle that causes a joint to remain in a bent position. In JDMS, there are two ways a contracture may occur:
Through the healing process: As the muscle heals, it may scar. These scars can shorten the muscle, and pull the joint into a bent position.
Through a lack of exercise: At times, your child's muscles might feel too sore to exercise. Without exercise, the muscles become weak. Because a weak muscle cannot hold the joint in its correct, extended position, a contracture will occur.
You and your child can help prevent serious joint
contractures with daily exercises and proper physical therapy.
When the body is fighting a disease, it uses a lot of the energy which it would normally use for growth. Because your child may not feel well, she may not feel like eating. These factors, along with some of the medicines used in treatment, may cause your child to grow and mature more slowly than normally. Usually, your child will begin to grow again once the disease is under control.
How is JDMS Diagnosed?
In some children, the signs of JDMS develop
over a long period of time. In others, the signs develop quickly. To find out
if your child has JDMS, the doctor will ask about your child's health history,
perform a complete physical examination, and obtain laboratory tests.
Child's Health History
The doctor will ask many questions about your child's symptoms, such as:
· When did the symptoms start?
· What parts of the body are involved?
· Are the symptoms always present, or present only at certain times?
· Are the symptoms getting better or worse?
· How are your child's activities affected by the illness
· Has she had any choking or regurgitation
· Can she carry her back pack and books
The doctor may also want to know if other
members of the family have had arthritis or a muscle disease since, in rare
instances, some forms of these illnesses may run in families.
The doctor will examine your child, paying special attention to skin and muscles. In particular, the doctor will look for these signs:
· a rash on the face, knuckles, elbows, knees, and/or cuticles
· swelling around the eyes
· nasal speech pattern
· sore and/or weak muscles
· sore and/or swollen joints
will carefully check your child's strength and will check routine areas, such
as the eyes, ears, nose, and throat. The strength exam is extremely important;
your doctor will ask your child to lift her arms, legs or head and then see if
she can hold them up against the doctor's resistance. Only with a proper muscle
exam can the weak muscles be identified as weak. Your child will develop all
kinds of movements to hide her disability. One of the things you should look
for in your doctor is his or her ability to show you your child's weakness.
Laboratory tests help determine if your child has JDMS. These tests show if there is inflammation in the muscles or in other areas of the body. Many of these tests may be abnormal early in the course of the illness, even if your child has only mild muscle weakness. The most common tests are:
When the muscles are inflamed or damaged, certain proteins from inside the muscle cells (called enzymes) leak into the blood stream and their levels can be measured. The doctor will look for these enzymes such as CPK and aldolase. Your doctor may also tell you about other enzymes, called AST, ALT and LDH. These are less specific than CPK and aldolase but are often used to assess how medications are working. If large amounts of these enzymes are found in the blood, then the doctor knows that some muscles have been damaged.
Antinuclear antibodies (ANA)
This test shows whether or not your child's immune system is producing antibodies against the body's cells. This is not a specific test for DMS, it is often positive in other rheumatic diseases.
This test measures electrical activity in the muscles. Children with JDMS have a specific electrical pattern that shows the muscle is inflamed.
In this test, a thin needle is placed through the skin and into the muscle. A wire runs from the needle to a machine that records electrical activity in the muscle. Unfortunately, it is painful and therefore it is not done frequently.
This test provides the most specific information about your child's inflamed muscles. It shows how much the muscles or blood vessels have been affected by the disease. If your child needs a biopsy, the doctor will remove a small piece of muscle and look at it and its surrounding blood vessels under a microscope to see if there is any inflammation.
Most doctors no longer perform EMG or muscle biopsy if the child has a typical rash, demonstrable muscle weakness during the physical examination, and elevated muscle enzymes in the blood test. However, if there is no rash, or muscle enzymes are normal despite a typical rash, EMG and/or muscle biopsy is needed.
MRI (magnetic resonance imaging)
MRI, a very sophisticated Xray, is sometimes done to find inflamed muscles in children who remain strong and have normal or just slightly elevated levels of muscle enzymes; the MRI helps identify muscles most suitable for biopsy.
By putting together the story of how the illness started with the results of the physical examination and the laboratory tests, the doctor can usually make the proper diagnosis.
What Is the Treatment for JDMS?
There is no cure for JDMS, but treatment can help prevent or control most serious problems. Your child's treatment plan will be based on the severity of the illness. The goals of any treatment program for JDMS are to:
· control muscle inflammation and damage
· maintain and improve muscle strength and function
· relieve pain
· control or prevent other symptoms
· help child and family learn to live with the illness.
To reach these goals, treatment usually includes:
· a balanced diet
As your child's symptoms change, the treatment
plan may also be changed.
There are several medications your doctor may use to treat JDMS. The specific drug or combination of drugs your doctor uses depends on the type and severity of your child's symptoms. Since only common side effects of each drug are listed here, you should discuss your child's medications with the doctor.
Prednisone (also known as: steroid, corticosteroid, cortisone, solumedrol, prednisolone)
Prednisone is by far the most common and most important of the medications used in autoimmune diseases. It works quickly to slow down the immune system and help control inflammation in the muscles and joints. This drug is similar to cortisone, a natural body hormone. When children take this medicine, their body stops producing the natural hormone and your child becomes dependent on the medication and cannot simply stop it. In order to allow the body to resume production of the natural hormone, your doctor will slowly lower the medication so the body can gradually make it again.
Steroids can be given as pills, or as a large intravenous infusion weekly, or both. Some pediatric rheumatologists prefer to give a large dose intravenously initially and repeat this dose several times while giving a lower daily dose. Other doctors give only pills, while another one may advise both. No matter how it is administered, steroid is very successful in controlling your child's inflammation.
Prednisone is used to:
· relieve muscle and joint pain
· improve muscle strength
· control fever
· control skin rash
Dose: At first, high doses of this drug are given until your child's muscle enzyme tests and strength improve. As your child gets better, the dose will gradually be lowered.
Common side effects: The side effects your child may have depend on how much medicine the child takes, and how long she has taken it. Common side effects include:
· increased appetite
· weight gain
· stretch marks on the skin
· round, full cheeks
· high blood pressure
· mood changes
Over a long period of time, the drug can also cause these problems:
· weak or damaged bones
· small cataracts
· slowed growth rate
· greater chance of developing diabetes, if it runs in the family.
Prednisone may cause other side effects, depending on the dose and length of time your child takes it. Discuss the side effects with your child's doctor. This drug is given because the benefits of controlling the JDMS outweigh the risks of side effects. The doctor will try to lower the dose as soon as possible to lessen the side effects, while keeping the disease under control.
A word about relapses: as the prednisone dose is lowered, your child's earlier symptoms of JDMS may return. This is called a relapse. These symptoms will usually go away with a mild increase of prednisone or by introducing another medication to be used with prednisone. Although some children may have more than one relapse, a relapse may not necessarily slow your child's recovery from JDMS.
If prednisone alone can't control your child's illness, a stronger drug such as Methotrexate is often used. This drug also slows down the body's immune system but it works completely differently from prednisone and has a different set of potential side effects.
Dose: The doctor will use as low a dose as possible and will usually use it in combination with prednisone. Methotrexate can be given as a pill or by injection; most doctors will recommend injection because the GI tract may be inflamed and prevent pills from being absorbed completely. Methotrexate is given once per week, while prednisone is taken daily. As your child gets better, the doctor will slowly reduce the dose of one or both of the drugs,usually one drug at a time.
Common side effects: Because these drugs add more potential side effects, they are used only if other medicines have not controlled the disease well enough. Talk to your doctor to decide whether the benefits of the drug outweigh the risk of its side effects.
Side effects may include:
· increased risk for serious infections
· nausea and vomiting
· mouth sores
· skin rash
· damage to some internal organs, such as the liver
· decreased number of blood cells
These side effects will go away when the dose is lowered or stopped completely.
These drugs may cause other, more serious side
effects. If your child is taking any of these drugs, the doctor will take
regular blood and urine tests to monitor the drug's effect on your child.
Hydroxychloroquine (hi-DROCKS-ee-CLOR-o-kwine) is a drug sometimes used to treat a severe rash in JDMS. The brand name of this drug is Plaquenil (PLA-kweh-nill).
Dose: This drug is given as a pill. Since it simply controls the rash, your child will also take other medicines for other symptoms.
Common side effects: there are no common side effects but rarely there may be:
· upset stomach
· eye problems
After a long time, Plaquenil may interfere with color vision. That is why an ophthalmologist (eye doctor who is an MD) should check your child's eyes every six months while your child is taking Plaquenil. If eye problems are detected early, they should go away when the medicine is stopped.
If methotrexate and prednisone don't fully control your child's DMS, your doctor will probably advise cyclosporine (CSA). This is another immunosuppressive drug developed for organ transplantation. It helps control DMS especially in combination with Methotrexate. These two agents together often allow the prednisone to be tapered away. CSA may cause:
· extra hair growth on face and arms
· thickening and swelling of gums
· kidney problems including high blood pressure
is reserved for children with severe DMS
and should be administered only by an expert in DMS, namely, the Pediatric
rheumatologist. Both, blood and urine must be monitored monthly.
Exercise is a very important part of JDMS treatment: in fact, it is nearly as important as medication. The purpose of regular exercise is to:
· keep muscles as strong and flexible as possible
· keep joints flexible
A physical therapist, who is part of your child's treatment team, will teach your child exercises to do at home. These exercises will be designed specifically for your child and will change depending on the activity of the disease. At first, your child's muscles may be weak and she may need help with the exercises. Later on, when your child won't need as much help, the exercises will be changed.
Exercises usually take about one hour per day.
It is important for your child to do the exercises regularly, but not to
overwork and tire her muscles. The therapist will try to combine some exercises
with your child's normal play activity.
A Balanced Diet
Although there is no diet that will cure JDMS, it is important for your child to eat a regular, balanced diet. Protein in your child's diet is important for normal growth and to repair damaged muscles because muscle is made of protein. Calcium is important for strong bones. However, because prednisone is used to treat your child, the diet must be low in salt and in sugar. Once, your child regains her strength, her diet should be low in fat and overall calories but it must stay balanced. If your child does not feel well and does not want to eat, try to keep her interested in foods by letting her help prepare meals, and by preparing many different foods. Meals prepared at home are much healthier than anything you buy ready made. Specifically, fast foods should be discouraged and meals out need to become special treats. A nutritionist can help plan a good diet for your child.
Your doctor, nurse and other health care workers can help teach you about JDMS. By learning about the illness and how to treat it, both you and your child will be able to take better care of the disease, answer people's questions and feel in more control over your lives.
Who Should Treat My Child?
Your child's regular doctor is the best person
to see when your child first becomes ill. But, because JDMS is hard to diagnose
and treat, the primary doctor will send your child to a specialist such as the
Pediatric rheumatologist, a doctor who specializes in childhood rheumatic
diseases. Pediatric Rheumatologists are Pediatricians who spent an extra three
years training in the area of Rheumatology and have taken examinations which
certify that he or she are the expert in the recognition and treatment of
chronic autoimmune conditions of children. No other physician has the
experience to safely treat your child and the national
The Health Care Team
Several other health professionals may be involved in your child's treatment program:
Remember: your child may not need to see all of these persons, and may need to see others only a few times.
· nurse - teaches your child about JDMS and how to take care of it. This includes teaching pain control and play therapy to help the child deal with the illness.
· physical therapist - tests muscle strength and designs exercises which strengthen large muscles (such as the arms and legs) and keep joints flexible. A physical therapist can also help your child regain lost coordination and movement.
· occupational therapist - teaches new ways to do daily activities such as eating, combing hair, and dressing, which may be more difficult for a child with JDMS. These activities strengthen smaller muscles (such as those in the hand) and help the child feel more independent.
· social worker - helps with the personal, emotional, family, or financial problems that may occur with JDMS.
Other persons such as a nutritionist, pharmacist,
psychiatrist, or psychologist, can help you handle special problems.
Finding Medical Care
To find out about these health professionals in your area, contact any of these organizations:
· your local Arthritis Foundation chapter
· a local children's hospital or medical center
· your county medical society
What About Emotions?
A chronic illness like JDMS affects everyone in
the family. At first, your child and other family members may have troubling
feelings about the disease and its effect on the family. Yet as your child's
health begins to improve, you will gradually feel less troubled about the
illness. Settling back into a normal routine will benefit everyone in the
What You May Be Feeling
Parents When you are first told your child has JDMS, you might feel shocked, numbed, or disbelieving. You might also feel guilty, and ask yourself if something you did or didn't do caused your child's JDMS. You are experiencing the grieving process because you have in fact "lost" your healthy child. It takes time to go through this process, weeks to months, and it is a necessary part of accepting your child's chronic illness and coping with it most effectively and productively.
The child with JDMS The child with JDMS may feel many different emotions. Children can feel "hurt" by an illness that isn't their fault, blame parents for the illness, adopt a "why me?" attitude, and indulge in self-pity or anger because of restrictions on activities. They may also resent other children who are well, including brothers and sisters. Talk to your health care team about how to best help your child.
Brothers and Sisters Other children in the family may feel left out because of the amount of time and attention the child with JDMS requires. Or they may feel guilty, as if their normal "bad thoughts" towards their brother or sister had somehow caused the illness. They may be afraid they will also get JDMS or that their ill brother or sister may die from the disease.
What Can You Do To Help Them? They key to dealing with these emotions is to talk about them with one another.
For Your Child with JDMS Talk to your child about how she feels about the illness. Allow her to express her anger about JDMS from time to time.
Encourage your child to develop her special talents. Also, expect her to behave as well as other children: for example, make sure that she is given household chores which she can complete.
Encourage your child to learn about JDMS and
her treatment program and, as she grows older, give her more responsibility in
following that program. Remember that your attitude toward the disease will
affect the way your child feels about the disease.
For Other Family Members Talk to your child's brothers and sisters about JDMS - let them express their feelings about the disease.
Encourage the family to treat the ill child as they
did before she became ill - but at the same time, to also remember that she
will need some special attention.
For You and Your Spouse Try not to overprotect your child, even though this is a natural desire. Your child might become too dependent if you do everything for her, or it you do tasks which she is capable of doing.
Don't be "blackmailed" into allowing activities that shouldn't be done, but compromise when you can. Being as consistent as possible will help your child learn what is expected.
Plan special time to spend alone with your spouse, or with the entire family. When your child first becomes ill, you may set aside relationships with other family members. It is important, however, to continue to talk to and spend time with all family members.
it is important for the whole family to fit JDMS into its regular life pattern
and to adapt to the illness rather than fight it. This means fitting your
child's treatment program into the family's regular daily schedule. It means
asking for special help from people when you need it. Adapting also means
talking (and crying) about JDMS now and then. Of course JDMS will make life
more difficult than it used to be - but accept this as a challenge rather than
Where Can You Go For More Help?
Sometimes, the stress of having JDMS may cause your child to develop emotional or behavioral problems that you cannot deal with alone. Other family members may also feel overwhelmed. Whatever the case may be, counseling or support groups can help work things out. Sometimes all that is needed is a talk with a sympathetic person, another family who has a child with a rheumatic disease, or a professional who is trained to help in these situations. The doctor, nurse, or medical social worker may be able to help you or refer you to other sources of help.
What About School?
Generally, children with JDMS should attend a regular school and should not be isolated from other children their same age. But because of JDMS your child may need special services, such as special transportation or supplies, to help her get along in a regular school.
How can you help your child avoid problems at school?
· Be informed
· Inform others
There are two federal laws which affect your child's rights in school:
· Section 504 of The Rehabilitation Act of 1973
This law forbids all federal programs from discriminating against disabled persons. It means your child should be able to receive services from all programs and agencies (including schools) that receive money from the federal government.
· Public Law 94-142 (The Education for All Handicapped Children Act)
This law says that every child has a right to a free public education, whether or not the child has a disability. It means you can work with your child's school to make sure she receives the services she needs in order to attend public school.
For more details about these laws see When Your
Student Has Arthritis, available from your local Arthritis Foundation chapter.
For More Help
If you have problems protecting your child's rights, you can contact the following organizations:
American Juvenile Arthritis Organization
1314 Spring Street, N.W.
The Arthritis foundation's national membership organization that focuses on the problems related to arthritis and rheumatic diseases in children.
Federation for Children With Special Needs
A national organization that helps children with special needs obtain an equal opportunity for education.
Dystrophy Association (MDA)
A national organization that provides support services for persons with neuromuscular disorders.
Talk to people at the school about your child's illness. Tell your child's teachers, the school nurse, and the principal about:
· the disease, JDMS
· how JDMS affects your child, and problems that may occur in school
· your child's treatment program, including medications taken during school hours
· how to reach you or your child's doctor, if necessary
What About the Future?
Most children have active JDMS for about two years. Since JDMS affects every child differently, some children may have active disease longer than two years, and some may have more severe symptoms than others. Younger children may have an easier course than adolescents. Some children regain their strength quickly and medications can be eliminated during the first or second year but others may have recurrences or may have the type of disease that does not easily respond to medications and needs to be treated for many years. It is impossible to predict how your child will respond, only time will tell. The most important thing is to have confidence in your child's doctor and to insist that your child receive care from a Board Certified Pediatric Rheumatologist.
JDMS is a treatable disease. Although most children recover completely from the disease, some children will have permanent muscle weakness and stiffness from it. Despite the challenges children with JDMS and their families face, the majority of these children grow up to lead a fulfilling, happy life.